Adrenal hyperplastic nodules in Wiedemann-Beckwith syndrome.
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Biomedical subjects
Publications and source records attributed to C Sotelo-Avila.
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Neonatal transfusion practices during 1989 of 452 institutions involved in transfusing infants were surveyed by questionnaire. Most respondents (77%) transfused fresh frozen plasma appropriately (ie, primarily to treat coagulation disorders). However, 11% stated that their most frequent use of fresh frozen plasma was solely to treat hypovolemia, a practice generally not recommended. Seventy-eight percent of respondents transfused platelets to treat bleeding infants with blood platelet counts of less than 50 x 10(9)/L; 84% gave platelets to sick, premature neonates with counts of less than 50 x 10(9)/L whether or not bleeding was evident. Only 35% of respondents transfused granulocytes for neonatal sepsis; most institutions used buffy coats isolated from units of blood--a product readily available, but of questionable efficacy when compared with leukapheresis granulocytes. Ninety-three percent of respondents provided blood components with low risk of transmitting cytomegalovirus: components from seronegative donors were used by 84%, leukocyte-reduced products by 6%, and a combination by 10%. Thirteen percent of respondents gave gamma-irradiated blood components to all and 46% gave them to some neonates to prevent graft vs host disease. Forty-one percent did not routinely irradiate. Ten percent of respondents used leukocyte reduction instead of gamma irradiation to prevent graft vs host disease, a practice currently not advocated. Thus, national transfusion practices for neonates are variable, controversial, and, occasionally, other than those usually recommended. Additional research and educational efforts are needed to ensure optimal transfusion therapy.
Neonatal blood component transfusion practices during 1989 were surveyed via a questionnaire developed by the Pediatric Hemotherapy Committee of the American Association of Blood Banks. Of 1790 questionnaires mailed, 452 were selected to form the database for this analysis because they were from institutions in which neonates were transfused. Nearly all institutions contained intensive care units directed by neonatologists and were involved in the management of high-risk infants. Results from institutions serving as the primary pediatric teaching hospital of a medical school were compared with those with no medical school affiliation. Thirty-six percent of primary pediatric teaching hospitals and 52% of hospitals with no medical school affiliation performed pretransfusion testing in excess of that required, resulting in additional blood loss in neonates. Sixty-six percent of primary pediatric teaching hospitals used fresh frozen plasma to adjust the hematocrit of red blood cell concentrates prior to transfusion (a practice increasing donor exposure), compared with only 29% of hospitals with no medical school affiliation. The usual indication for small-volume red blood cell transfusions in severely ill neonates was to maintain a desired hematocrit level, whereas for stable infants, red blood cell transfusions were given to treat symptomatic anemia, rather than to maintain a predetermined hematocrit. As found in 1985, neonatal transfusion practices in 1989 were variable. However, improvements have occurred since 1985 to suggest that further research and educational efforts may serve to promote even better neonatal transfusion therapy.
The Kowa FC-1000 laser flare-cell meter (LFCM) has been described as an instrument which will objectively quantify inflammation of the anterior chamber of the eye. We evaluated the LFCM using the intravenous endotoxin-induced uveitis (EIU) rabbit model of ocular inflammation. In vitro flare and cell calibration measurements utilizing bovine serum albumin (BSA) and latex particles, respectively, were also performed. A linear relationship between the flare measurements and BSA concentrations was noted. In addition, the time course of the LFCM flare count in EIU was comparable to previously published fluorophotometric data. However, the LFCM reported cells in the anterior chamber of the EIU rabbits despite negative cytology and histology results. The LFCM also recorded cells in BSA solutions which contained neither cells nor latex particles. Our results suggest that although the LFCM may be useful for evaluating flare, its cell measurements are not accurate in cases of severe uveitis.
Partial hepatectomy (PH) initiates cellular signals for regeneration. Sequential expression of nuclear and cytosolic protooncogenes accompanies the restoration of normal liver function and architecture. Although cirrhosis is known to inhibit liver regeneration, the effects of noncirrhotic cholestasis on hepatocellular proliferation, differentiation, and regulatory gene expression are unknown. To examine this, 25 male Fisher rats underwent common bile duct ligation and division. A 47% +/- 5% PH was performed 10 days after common bile duct ligation and division when histologic analysis revealed cholestasis without cirrhosis. Despite early elevations of total hepatic DNA and RNA values, cholestatic livers demonstrated a significant threefold suppression of expected hepatocyte mitotic indexes 48 and 72 hours after PH, compared with livers after PH alone. Weight restoration in cholestatic livers was 11% +/- 5.2% compared with 40% +/- 4.3% in control livers (+/- SEM; p less than 0.001) 5 days after PH. Analysis of regenerating liver messenger RNA with complementary DNA probes revealed an abnormal, sustained elevation of K-ras expression in cholestatic livers through all time points. Cholestasis blunted but did not obliterate normal sequential elevations in H-ras found in control livers. The expression of c-myc was inhibited threefold with cholestasis 72 hours after PH. These results are the first indication that cholestasis alone inhibits hepatocyte proliferation and the expression of c-myc that normally precedes the first wave of mitosis. This implies that cholestasis without cirrhosis may alter programmed liver gene expression, inhibiting normal hepatic regeneration.
Cutaneous mucinosis is an uncommon lesion characterized by mucin accumulation in the dermis. Rarely, mucinosis may arise in the oral cavity; reported cases have been observed in patients older than 16 years. We identified two children younger than 5 years of age with focal mucinosis of the upper aerodigestive tract. A 3-year-old boy had been intubated at age 2 months, suffered from recurrent respiratory infections, and developed mucinosis of the larynx, a previously unreported site for this unusual lesion. The second patient, a 4-year-old girl with a cleft palate, developed palatine mucinosis.
Ureterosigmoidostomy, frequently performed in children for urinary diversion, is a known premalignant condition. Dietary calcium supplements have been shown to normalize mucosal proliferative patterns in other human premalignant colonic conditions, which might decrease the risk of cancer. However, calcium supplementation has not been investigated in ureterosigmoidostomy. We used a rat model to study the effects of increased dietary calcium on the progression to carcinoma in ureterosigmoidostomy. Twenty-five Wister rats underwent ureterosigmoidostomy by anastomosis of bladder trigone and ureters to sigmoid, and were divided into two groups: group 1, regular diet (14); and group 2, calcium supplemented (2%) diet (11). All animals received the diet for 9 months. At death the anastomosis was weighed and examined histologically. In addition, ornithine decarboxylase (ODC), an enzyme involved in polyamine synthesis that becomes elevated in neoplastic and proliferative disorders, was determined at the anastomosis and 8 cm proximal (normal colon). All animals developed proliferative metaplastic polyposis at the anastomosis. Tumor weights were 2.6 +/- 1.1 g for group 1 and 4.8 +/- 1.2 g for group 2 (P less than .05). The ODC levels were: group 1, normal colon 78.2 +/- 15.6 and tumor 321.3 +/- 58.8 (P less than .002); and group 2, normal colon 425.8 +/- 65.4 and tumor 568.0 +/- 40.9 (P = NS). The difference between group 1 normal colon and group 2 normal colon was significant (P less than .001), as was group 1 tumor and group 2 tumor (P less than .02).(ABSTRACT TRUNCATED AT 250 WORDS)
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Cholecystitis and cholelithiasis are infrequent in children and have been historically associated with adolescent pregnancy or hemolytic disorders; however, the incidence and spectrum of cholelithiasis seem to be changing. Between 1970 and 1988, 47 children 17 years of age or less underwent cholecystectomy for cholecystitis or cholelithiasis in our hospital. The patients were divided into chronologic groups: Group 1 encompassed 1970 through 1979 (15 patients) and group 2, 1980 through 1988 (32 patients). The groups were compared for age, sex, pregnancy, blood dyscrasia, family history, obesity, use of total parenteral nutrition (TPN), and incidence of choledocholithiasis with its sequelae. A significant increase in the number of patients with cholelithiasis was found. Infants and young children were affected more frequently in group 2, and many of these young patients had a history of TPN. Choledocholithiasis was also more common in group 2 and presented with life-threatening sequelae. Calculous biliary tract disease should be considered as a possible cause of abdominal pain in children. Timely operative intervention can prevent the increasingly common sequelae of childhood cholelithiasis.
We found fibrin thrombi or thromboemboli at autopsy in 22 of 23 infants with respiratory failure who had been treated with venoarterial extracorporeal membrane oxygenation (ECMO). In addition, distinctive basophilic aluminum-containing emboli were found in 12 of the infants; the distribution of these emboli was similar to that of the thromboemboli, except that an aluminum-containing embolus was found in a lung in only 1 infant. Sixteen infants had pulmonary thrombi or thromboemboli. We also found friable aluminum-containing concretions adhering loosely to the mixing rods of heat exchangers that had been used to warm the blood flowing through the ECMO circuit; such concretions were not present on unused mixing rods. We propose that these aluminum-containing concretions developed as the silicone coating of the heat exchanger wore away and aluminum metal was exposed to warm, oxygenated blood and that fragments of aluminum-containing concretions formed emboli. This hypothesis is supported by the fact that aluminum-containing emboli were generally not present in the lungs, which are bypassed by ECMO. Although infarcts were found in 16 of the 23 infants, we cannot be certain whether any of the infarcts were caused by the aluminum-containing emboli.
Urinary diversion into the gastrointestinal tract (ureterosigmoidostomy) is associated with stepwise malignant degeneration of colonic mucosa. Early detection of such malignancy can be difficult. Ornithine decarboxylase (ODC) is an enzyme that initiates polyamine synthesis that is elevated in malignant colonic mucosa, but its level in premalignant mucosa after ureterosigmoidostomy is unknown. Ten Wistar rats underwent urinary diversion (bladder trigone to sigmoid colon), and were maintained on a regular diet with antibiotics for 6 months, then killed. All animals developed metaplastic changes histologically at the anastomosis. Mean ODC levels of colonic mucosa at the anastomosis v normal colon 8 cm proximal were 515 +/- 177 pmole v 24.5 +/- 4.4 (P less than .01). These data show that premalignant changes in colonic mucosa after ureterosigmoidostomy can be detected by elevated colonic biopsy ODC levels. Periodic sigmoidoscopy with colon mucosa biopsy for histology and ODC levels in children with ureterosigmoidostomy is recommended.
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This is a report of the histochemical and ultrastructural analysis of nevus cell aggregates in the placenta from a neonate with a giant pigmented nevus. Although the distinction between aberrant migration of neural crest elements and blood-borne metastasis from the giant pigmented nevus is not readily resolved, the authors favor the former as an explanation of this unusual placental lesion.
A case of malignant rhabdoid tumor of the spine and a description of its CT findings are presented. The CT demonstrated a heterogeneous contrast enhancing epidural mass associated with severe bony destruction of the adjacent vertebra and cord compression.
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In a retrospective review, we identified a variety of abnormal immature and differentiated metanephric tissues including nodular renal blastema and metanephric hamartomas in 12 children with urinary tract malformations with or without renal dysplasia. Four infants also had trisomy. Nodular renal blastema was characterized by small subcapsular clusters of primitive metanephric cells, in some cases with evidence of tubular, papillary, or trabecular differentiation. Metanephric hamartomas included papillary adenomas and tubular hamartomas. Undifferentiated renal blastema was multifocal, but more differentiated blastema usually occurred as isolated nodules. When accompanied by renal dysplasia, blastema tended to show differentiation. One child had a spectrum of lesions with both blastema and more differentiated metanephric hamartomas.
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