Biomedical subjects
Publications and source records attributed to C K Chen.
Complications resulting from duodenal diverticula are relatively uncommon. We describe small bowel obstruction secondary to an enterolith formed in a duodenal diverticulum, probably as a result of habitual ingestion of large amounts of kaolin.
In an experimental model of perinatal hypoxic-ischemic brain injury, we examined quisqualic acid (Quis)-stimulated phosphoinositide (PPI) turnover in hippocampus and striatum. To produce a unilateral forebrain lesion in 7-day-old rat pups, the right carotid artery was ligated and animals were then exposed to moderate hypoxia (8% oxygen) for 2.5 h. Pups were killed 24 h later and Quis-stimulated PPI turnover was assayed in tissue slices obtained from hippocampus and striatum, target regions for hypoxic-ischemic injury. The glutamate agonist Quis (10(-4) M) preferentially stimulated PPI hydrolysis in injured brain. In hippocampal slices of tissue derived from the right cerebral hemisphere, the addition of Quis stimulated accumulation of inositol phosphates by more than ninefold (1,053 +/- 237% of basal, mean +/- SEM, n = 9). In contrast, the addition of Quis stimulated accumulation of inositol phosphates by about fivefold in the contralateral hemisphere (588 +/- 134%) and by about sixfold in controls (631 +/- 177%, p less than 0.005, comparison of ischemic tissue with control). In striatal tissue, the corresponding values were 801 +/- 157%, 474 +/- 89%, and 506 +/- 115% (p less than 0.05). In contrast, stimulation of PPI turnover elicited by the cholinergic agonist carbamoylcholine, (10(-4) or 10(-2) M) was unaffected by hypoxia-ischemia. The results suggest that prior exposure to hypoxia-ischemia enhances coupling of excitatory amino acid receptors to phospholipase C activity. This activation may contribute to the pathogenesis of irreversible brain injury and/or to mechanisms of recovery.
Solid and papillary epithelial neoplasms of the pancreas are uncommon. These grow to a large size, are often palpable, and occur most often in young black women. The tumors have a characteristic histologic appearance and a low malignant potential. Heavy tumor calcification is an unusual finding. Two cases with radiologic-pathologic correlation are presented herein.
It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. In 16 affected children, only 4 (25%) had minimal-change lesion, 7 had IgM nephropathy, 3 had diffuse mesangial hypercellularity, and 2 had focal segmental glomerulosclerosis. Fourteen of these patients have required immunosuppressive therapy with cyclophosphamide. Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.
Balloon coarctation angioplasty (BCA) was performed in seven consecutive patients (five boys and two girls) 18 months to 18 years old (mean 9.5) with isolated discrete unoperated coarctation of the aorta. A No. 8F or 9F catheter was chosen with balloon lengths of 30 or 40 mm and maximum inflation diameters 1 mm less than the smallest measured aortic diameter determined 1 cm proximal to the coarctation site. A 10 sec inflation-deflation cycle of 6 to 8 atmospheres (90 to 120 psi) was performed. The peak systolic pressure gradient (PSG) before BCA ranged from 35 to 70 mm Hg (mean 58), and immediately after BCA it decreased to 0 to 20 mm Hg (mean 7). One to two year follow-up (mean 14 months) of the seven patients revealed a PSG range of 10 to 30 mm Hg (mean 19). Repeat angiography was performed immediately proximal to the coarctation site. Three patients (43%) had evidence of aneurysm formation at or immediately distal to the balloon dilatation site. One patient had coarctation restenosis. While initial results with BCA for unoperated coarctation were encouraging, current data raise serious concerns about its long-term safety and efficacy.
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Sickle cell nephropathy characterized by proteinuria and predominantly glomerular lesions has not been studied as extensively as renal tubular alterations in sickle cell disease. We reviewed our experience with this entity over a 14-year period. Of 13 children with either proteinuria or the nephrotic syndrome, 8 showed focal and segmental glomerulosclerosis, and 5 had mesangial proliferation. Children with focal and segmental glomerulosclerosis were older at onset of nephropathy and presented with the nephrotic syndrome more frequently than those with mesangial proliferation (p less than 0.05). All patients with mesangial proliferation and half of the focal and segmental glomerulosclerosis patients had supranormal renal clearances at onset of nephropathy suggesting hyperfiltration. Hyperfiltration seen in animals with reduced renal mass, and in human diabetic nephropathy before reduction in nephron units leads to mesangial proliferation and sclerosis. Our study suggests that sickle cell disease produces similar lesions in patients with sickle cell nephropathy.
We have reviewed the efficacy of cyclophosphamide in 39 steroid-sensitive frequently relapsing nephrotic children. Cyclophosphamide was used because of heavy steroid dependence and steroid toxicity. A percutaneous renal biopsy done prior to administration of cyclophosphamide showed the lesion to be minimal change in 7 children, IgM nephropathy in 17 children, and evolving from minimal change nephrotic syndrome to focal segmental sclerosis (FSGS) in 15 children. 100% of patients with minimal change nephrotic syndrome responded to cyclophosphamide, but only 58% of IgM patients responded (p less than 0.05). Only 1 of 15 FSGS patients responded (p less than 0.001 vs. minimal change nephrotic syndrome and p less than 0.01 vs. IgM). In view of the failure of cyclophosphamide to produce a remission in FSGS and its potential for long-term impairment of suppressor T cell function, we suggest that it should not be used in patients whose disease has evolved from minimal change to FSGS.
Of the 92 patients with the acquired immunodeficiency syndrome (AIDS) who were seen at our institution over a two-year period, 9 acquired the nephrotic syndrome (urinary protein greater than 3.5 g per 24 hours) and 2 had azotemia with lesser amounts of urinary protein. Five of these 11 patients had a history of intravenous-heroin addiction, but in the remaining six, there were no known predisposing factors for nephropathy. In nine patients (including the six non-addicts) the course of renal disease was marked by rapid progression to severe uremia. Renal tissue examined by biopsy in seven patients and at autopsy in three revealed focal and segmental glomerulosclerosis with intraglomerular deposition of IgM and C3. In the 11th patient, renal biopsy revealed an increase in mesangial matrix and cells, with deposition of IgG and C3 consistent with a mild immune-complex glomerulonephritis, and severe interstitial nephritis. We conclude that focal and segmental glomerulosclerosis may be associated with AIDS and suggest that rapid deterioration to uremia may characterize this renal disease.
A 9-year-old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which re-resulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported before.
In two patients with systemic lupus erythematosus, acute renal failure was the initial manifestation. The diagnosis was eventually established on the basis of serologic tests and characteristic renal histopathologic findings. We emphasize the need to consider systemic lupus erythematosus as a cause of acute renal failure of glomerular origin, because appropriate therapy may alter the outcome of the disease.
We studied the long-term outcome of lupus nephritis in 23 black and Hispanic children. The follow-up period ranged from two to 16.5 years, with a mean follow-up of 5.4 years. The mean age at onset was 10.1 years, which is younger than that described in recent series of children with lupus nephritis. All patients had renal involvement, including four normotensive patients with normal renal function and normal urinary sediment. When children whose disease started before the age of 10 years were compared with patients older at onset, there were no significant differences regarding the type of lesion or duration of therapy, but a higher incidence of renal death (.02 less than P less than .05) was noted in younger children. Overall, 25% of our patients have died of renal causes, and another 25% have been undergoing dialysis, receiving transplants, or in chronic renal failure. The mortality in our series was higher than that reported in other series of children with lupus nephritis in recent years. Age and race may be acting synergistically to produce the higher mortality and morbidity.
The epithelium of the surface mucosa of the human stomach is demonstrated to share an antigen (HP-DU-1) with human pancreatic ductal cell surface epithelium detectable by a murine monoclonal IgG. This marker was found to be characteristic of the epithelium of gastric surface mucosa and serves to distinguish these cells from the epithelium of gastric glands, the generative cell zone, the parietal and mucous neck cells. The absence of HP-DU-1 was confirmed in the epithelium of the small and large intestines, gall bladder, tracheobronchial trees, urinary bladder, intrahepatic bile ducts, prostatic and salivary glands. This surface marker was used to examine the participation of the surface mucosal cell in hyperplastic, pre-neoplastic and neoplastic lesions of the human gastric mucosa. Gastric hyperplastic polyps and polypoid hypertrophic gastritis were mainly composed of epithelium bearing HP-DU-1 antigens. In contrast epithelial cells of atrophic gastritis, atrophic gastritis with intestinal metaplasia, and adenocarcinoma of the stomach lacked this antigen.
We studied the long-term outcome of a group of children with the nephrotic syndrome who showed the histological lesion of focal segmental glomerular sclerosis (FSGS) during the course of their illness. Of 25 such children studied, a complete follow-up ranging from 3 to 19 years was available in 24. Two distinct groups could be identified. Patients in the first group were characterized by steroid resistance (SR) from the onset, whereas those in the second group were initially steroid sensitive (SS), and had the histological lesion of minimal change which, over time, evolved into FSGS. SR patients had a mean age of 7.7 +/- 3.7 years compared to SS patients who were 3.5 +/- 2.5 years old (p less than 0.01). There were more females (11 of 14) in the SR group than in the SS group (3 of 10; p less than 0.02). The incidence of hematuria was higher in the SR patients (9 of 14) than SS patients (2 of 10; p less than 0.05). SR patients also exhibited a greater degree of growth retardation at the end of the follow-up period (9 of 13 compared to 1 of 8 SS patients; p less than 0.02). SR patients reached end-stage renal failure earlier (2.3 +/- 1.3 years) than SS patients (10 +/- 5.8 years; p less than 0.01) after the initial biopsy. Of the 13 kidney transplanted into 9 SR patients, recurrence of FSGS was noted in two allografts. Of the 4 kidneys transplanted into 2 SS patients, recurrence was seen in 1. The overall recurrence rate of FSGS in allografts was 17.6%. Our study suggests that the two varieties of FSGS occurring in nephrotic patients may be distinct nosologic entities rather than a single disease with varied manifestations.
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We have described a case of oncocytic carcinoma arising in the nasal mucosa which had invaded the maxillary and ethmoidal sinuses. Seven years after local resection with postoperative low-dose irradiation, the tumor recurred as a poorly differentiated adenocarcinoma. It is our opinion that oncocytic neoplasms arising in the nasal mucosa and demonstrating aggressive infiltrative growth should be regarded as malignant neoplasms, albeit sluggishly metastasizing ones, despite their deceptively benign histologic appearance.
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Most colonic polyps in children are of the juvenile type and occur either as single or scattered colonic polyps. The peak incidence occurs between 4 and 6 yr of age, with a spontaneous decline from 12 to 15 yr. Significant clinical symptoms are rare, and operative therapy is rarely indicated. Diffuse colonic juvenile polyposis, however, varies with different clinical, prognostic, and genetic implications. In infancy, colonic polyposis may be associated with diffuse gastrointestinal involvement leading to fatal complications unless treated aggressively. In childhood, colonic polyposis can occur with a genetic variance with an increased incidence of familial intestinal malignancies. Colonic polyposis in childhood, both familial and nonfamilial, can present with a mixed form of juvenile and adenomatous polyposis. In children with colonic polyposis, the biopsy of a single polyp that reveals the histologic appearance of a juvenile polyp does not rule out the simultaneous existence of adenomatous polyps.