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Biomedical subjects

R Superina

Publications and source records attributed to R Superina.

At least 19 recordsLinked to original sources
PubMed2001

Utility of semiquantitative polymerase chain reaction for Epstein-Barr virus to measure virus load in pediatric organ transplant recipients with and without posttransplant lymphoproliferative disease.

We examined the utility of Epstein-Barr virus (EBV) load as a test for the presence of posttransplant lymphoproliferative disease (PTLD). A semiquantitative (SQ) EBV polymerase chain reaction (PCR) on peripheral blood mononuclear cells (PBMC) was used to determine virus load. We compared the values from pediatric patients, both with and without PTLD, with those from healthy pediatric and adult subjects. The virus loads for asymptomatic healthy subjects had a range of 0-1 log10 cells/10(6) PBMCs. Among transplant recipients (n=135), the mean virus load (+/- standard deviation) at the time of diagnosis of PTLD was 3.1+/-1.2 log(10) cells/10(6) PBMCs versus a baseline value of 1.3+/-1.4 log(10) cells/10(6) PBMCs in children without PTLD (P<.0001). A cutoff of > or =3 log10 cells/10(6) peripheral blood leukocytes resulted in the following values for use of virus load as a test for PTLD: sensitivity, 69%; specificity, 76%; positive predictive value, 28%; and negative predictive value, 95%. We conclude that determination of EBV load by use of SQ PCR is more useful in ruling out than in indicating the presence of PTLD.

Adult↗
PubMed2001

Body mass index as a predictor of hepatic steatosis in living liver donors.

Evaluation of the living donor for liver transplantation is a complex process involving such invasive studies as liver biopsy and angiography. It is important to establish the likelihood and extent of hepatic steatosis in living donors by clinical, imaging, and biochemical parameters to avoid performing a liver biopsy, if possible. In this study, the predictive value of body mass index (BMI), liver chemistry tests, and imaging studies was compared with liver histological examination in 33 potential living donors. Patients were grouped and compared based on their BMI (<25, 25 to 28, >28). No patient with a BMI less than 25 had hepatic steatosis. Of patients with a BMI of 25 to 28, steatosis was found on biopsy in 3 of 9 patients. Thirteen of 17 patients (76%) with a BMI greater than 28 had hepatic steatosis on liver biopsy. There was a significant correlation between BMI and overall grade of steatosis (R = 0.49). All subjects with steatosis detected on magnetic resonance imaging (MRI) or computed tomography (CT) had steatosis on biopsy, and all but 2 such patients had greater than 10% steatosis on biopsy. Conversely, 30% of patients in the MRI group and 24% of patients in the CT group failed to show hepatic steatosis when it was present on biopsy. Thus, it appears that liver biopsy could be avoided in subjects with a normal BMI and absence of risk factors. Individuals with a high BMI should undergo liver biopsy because biochemical and imaging data are currently inadequate to determine the extent of steatosis. Future studies should aim at improving the sensitivity of imaging techniques in the diagnosis of steatosis.

Adolescent↗
PubMed2000

Experience with the Rex shunt (mesenterico-left portal bypass) in children with extrahepatic portal hypertension.

BACKGROUND/PURPOSE: Extrahepatic portal vein thrombosis (EPVT) in children can lead to severe bleeding from gastrointestinal varices, ascites, thrombocytopenia from hypersplenism, and other coagulation disorders. The authors have used the superior mesenteric vein to intrahepatic left portal vein (Rex) shunt in 5 children with symptomatic EPVT and report their results with this novel technique. METHODS: Children with symptomatic portal hypertension were screened for the underlying cause. All children with essentially normal livers and obstruction of the extrahepatic portal vein were considered for the Rex shunt. Evaluation included liver function tests, liver biopsy, and radiological evaluation of the intrahepatic vascular anatomy. RESULTS: Five patients between the ages of 2.8 and 10.5 years underwent evaluation for portal hypertension secondary to extrahepatic portal vein obstruction. Three patients had idiopathic extra hepatic portal vein thrombosis with cavernous transformation, 1 had thrombosis after a living-related liver transplant, and 1 had compression and obstruction of the main portal vein from enlarged lymph nodes after treatment of systemic histoplasmosis. All patients were symptomatic. Three patients had intermittent bleeding from esophageal and gastric varices, and all 5 had relative degrees of hypersplenism with enlarged spleens and thrombocytopenia (11,000 to 77,000). Three patients had significant leukopenia. Results of imaging studies suggested that 3 patients had inadequate intrahepatic portal veins for shunting, but all patients at exploration underwent successful shunting. There were no serious intraoperative complications. Postoperative complications included ascites in 2 patients that resolved within 1 month. There were no early shunt thromboses. The median postoperative length of stay was 7 days. Clinical follow-up ranged from 7 to 21 months. Gastrointestinal bleeding did not recur in any patient, and ascites resolved in all. Spleen size decreased significantly (P < .01) from 9.4 +/- 4.0 cm to 5.0 +/- 3.7 cm below the left costal margin. Mean platelet count and white blood cell count rose after shunting from 79 +/- 42 to 176 +/- 73 (P < .02) and 5.4 +/- 2.3 to 7.5 +/- 3.9 (P = .06), respectively. All shunts were studied at 1 and 7 days, and 3 and 6 months after the procedure. Shunt patency was documented in all cases. Subsequently, shunt blockage occurred in 2 patients. CONCLUSIONS: The Rex shunt has proven to be an effective method of resolving portal hypertension caused by EPVT including thrombosis after living donor transplantation. This shunt is preferable to other surgical procedures because it eliminates portal hypertension and its sequelae by restoring normal portal flow to the liver.

Child↗
PubMed1999

Lymphoproliferative disorders after organ transplantation in children.

BACKGROUND: After organ transplant, patients are at risk of posttransplant lymphoproliferative disorders (PTLD). The purpose of this study was to analyze 26 pediatric cases of PTLD observed at our institution between 1988 and 1996, and to evaluate the validity of the Society for Hematopathology Workshop (SHPW) 1997 classification in our patient population. METHODS: Charts were reviewed for analysis of incidence, clinical course, and outcome. Tissue samples were classified by a pathologist according to SHPW recommendations. RESULTS: By morphology, 20 were monomorphic, 5 polymorphic, and 1 hyperplastic. Assessment of lineage by morphology, molecular studies, and immunophenotyping did not correlate in six cases. By immunophenotyping, 12 were B cell, 4 T cell, 8 mixed B/T cells, and 2 undetermined. The 20 patients evaluable for treatment efficacy were treated with various therapeutic combinations, including immunosuppressive drug reduction, acyclovir/ganciclovir, interferon-alpha, immunoglobulins, surgery, and local irradiation. No patient received systemic chemotherapy. Thirteen patients achieved complete remission and 3, partial; 1 died 5 days after starting therapy, and 3 of progressive disease. Adverse prognostic factors included low platelet or neutrophil counts; stage III-IV and SHPW morphology were marginally significant. CONCLUSIONS: The majority of patients eligible for treatment can be cured with immunosuppressive drug reduction and antiviral drugs, along with surgery and irradiation when indicated. Systemic chemotherapy or innovative approaches may have a role in unresponsive cases. Morphologic SHPW grouping is feasible and seems to have clinical relevance. However, correlation with clonality and immunophenotyping is not always possible, necessitating modifications including segregation of descriptive morphology from clonality and cell origin.

Adolescent↗
PubMed1998

Experience with papillary and solid epithelial neoplasms of the pancreas in children.

BACKGROUND: Papillary cystic tumour of the pancreas in children is a rare tumour. METHODS: Ten patients admitted to The Hospital for Sick Children, Toronto, have been reviewed, and presentation and management are documented. RESULTS: One patient who had disseminated disease at presentation died. CONCLUSION: Excision of the tumour irrespective of size is recommended.

Adolescent↗
PubMed1998

Outcome after ileoanal anastomosis in pediatric patients with ulcerative colitis.

BACKGROUND: To review the outcome after restorative proctocolectomy among children and adolescents with ulcerative colitis at a pediatric inflammatory bowel disease center. METHODS: The records of all patients with ulcerative colitis undergoing colectomy and ileoanal anastomosis at The Hospital for Sick Children, Toronto, Canada, were reviewed. Questionnaires concerning functional results were sent to patients with restored transanal defecation. RESULTS: Seventy three patients (mean age, 13.2 years; range, 2.6-18.8 years) underwent ileoanal anastomosis (19 straight ileoanal anastomosis, 41 J pouch, 13 S pouch) between January 1980 and June 1995 and were observed 5.8+/-3.3 years. The ileoanal anastomosis is nonfunctional in 19 (26%) patients. Excision rates according to type of restorative procedure were J pouch, 7% (3 of 41); S pouch, 32% (4 of 13); and straight ileoanal anastomosis, 32% (6 of 19). Failure was usually attributable to intractable diarrhea among patients with straight ileoanal anastomosis but was caused by anastomotic leak or pelvic-perianal sepsis among patients with pouch procedures. Failure rates did not vary with age at ileoanal anastomosis. Among patients retaining ileoanal continuity, continence problems reported in the questionnaire were frequent and tended to be more extreme among younger patients. Overall, 90% of respondents reported satisfaction with the functional outcome of the restorative operation. CONCLUSIONS: The success rate of the ileoanal anastomosis/J-pouch procedure is comparable to that in adult series. The ileoanal anastomosis/J-pouch procedure is the operation of choice for children and adolescents who want ileoanal continuity restored after colectomy for ulcerative colitis.

Adolescent↗
PubMed1997

Laparotomy or drain for perforated necrotizing enterocolitis: who gets what and why?

Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However, for neonates less than 1,000 g survival in the drained group was 69% compared to 22% for the laparotomy group (P <.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

Journal Article↗
PubMed1997

Hirschsprung disease, postaxial polydactyly, and atrial septal defect.

We report on an infant girl with Hirschsprung disease, postaxial polydactyly, and atrial septal defect who was born to a consanguineous Iraqi couple. A similar condition of aganglionic megacolon, postaxial polydactyly, and ventricular septal defect with a presumed autosomal recessive (AR) inheritance was reported by Laurence in two sibs [Laurence et al.; J Med Genet 12: 334-338, 1975].

Abnormalities, Multiple↗
PubMed1997

The appearance of inverted Meckel diverticulum with intussusception on air enema.

This paper presents the appearances of inverted Meckel diverticulum with an irreducible intussusception on air enema in four children. The inverted Meckel diverticulum appeared as a bulbous (3) or triangular (1) filling defect in the air column projecting off the distal end of the soft tissue mass of the irreducible intussusceptum. The bulbous defect appears to be highly suggestive, and may be specific, for inverted Meckel diverticulum. Earlier recognition of the presence of the Meckel diverticulum as the lead point of the intussusception could have changed the management in two of the children.

Air↗
PubMed1997

Randomized comparison of ganciclovir plus intravenous immune globulin (IVIG) with IVIG alone for prevention of primary cytomegalovirus disease in children receiving liver transplants.

A randomized placebo-controlled trial was conducted to determine the benefit of ganciclovir (5 mg/[kg x d]) for 30 days in addition to intravenous immune globulin (IVIG) for 16 weeks for prevention of primary cytomegalovirus (CMV) disease in children receiving liver transplants. Patients were monitored for 6 months after transplantation. The two groups of patients (recipients of 29 ganciclovir plus IVIG and 27 recipients of IVIG alone) were similar in terms of age, sex, and underlying disease. The incidence of CMV disease among the ganciclovir plus IVIG recipients and the IVIG alone recipients was 17% and 26%, respectively, and the time to disease in these recipients was 46 days and 32 days, respectively. There was no difference between groups in terms of survival; episodes of rejection, bacteremia, or fungemia; use of immunosuppressive agents; and incidence of leukopenia or thrombocytopenia. These results suggest that a 4-week course of ganciclovir with IVIG is not more effective than IVIG alone for prevention of primary CMV disease. Since short-term prophylaxis with ganciclovir may delay the onset of CMV disease, further studies with a longer course of ganciclovir prophylaxis are warranted.

Adolescent↗
PubMed1997

Laparotomy or drain for perforated necrotizing enterocolitis: who gets what and why?

Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However, for neonates less than 1,000 g survival in the drained group was 69% compared to 22% for the laparotomy group (P <.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

Drainage↗
PubMed1996

Results of liver transplantation in children with unresectable liver tumors.

Liver cancer is an uncommon indication for liver transplantation in children. Between 1986 and 1995, five children with hepatocellular cancer (HCC), three with hepatoblastoma (HEP), and one with sarcoma were referred to the transplant service. All nine tumors were considered unresectable. Four of the five children with HCC had underlying predisposing conditions (2 hepatitis B, 1 biliary atresia, 1 tyrosinemia). Preoperative evaluation of all patients included careful radiological screening and pretransplantation laparotomy for staging. Two patients with HCC were excluded from further consideration because of intraabdominal spread. Three patients had transplantation (mean age, 6.0 +/- 7.1 years), and all have survived for 1 to 5 years with no evidence of recurrence. Three patients with HEP were assessed (mean age 2.0 +/- 1 years); two had stage 4 disease and one had stage 3. All three received preoperative chemotherapy. The two with stage 4 had thoracotomies as part of their assessment. Two of three patients had a significant decrease in the size of the primary tumor during the waiting period. These two patients and one with stage 4 disease have survived more than 2 years since transplantation, with no recurrence. The third patient had recurrence within 2 months of transplantation. In summary, liver transplantation should be considered for all children who have unresectable hepatic malignancies, given the 83% survival rate and no evidence of tumor recurrence. Stage 4 disease in HEP does not necessarily exclude patients from transplantation. Early referral is encouraged so that tumor spread beyond the liver is minimized.

Adolescent↗
PubMed1995

Fibrin clot lysis by tissue plasminogen activator (tPA) is impaired in plasma from pediatric patients undergoing orthotopic liver transplantation.

Large vessel thrombi can present life-threatening complications following orthotopic liver transplantation (OLT) in pediatric patients. We investigated the thrombolytic response to tissue plasminogen activator (tPA) of stored, pooled plasma (days 4-14 postoperatively) from 41 patients (mean age 4 years, 9 months) who underwent OLT at the Hospital for Sick Children, Toronto between 1986 and 1990. Trace-labeled fibrin clots were prepared by recalcifying 500-microliters aliquots of patient plasma spiked with 125I fibrinogen and then incubated at 37 degrees C in patient plasma in the presence or absence of tPA (0.1 or 0.3 mg/ml). At the end of the incubation period, the extent of clot lysis and concentrations of fibrinogen, plasminogen, and alpha 2 antiplasmin were determined. Pooled adult plasma was used as a control. Fibrin clot lysis in OLT plasma was significantly reduced compared with controls (P < 0.01). Initial concentrations of plasminogen were significantly reduced in OLT plasma. To determine if the low plasminogen levels limited the thrombolytic effect of tPA, we supplemented OLT plasma with purified plasminogen. Fibrin clots placed in OLT plasma containing adult levels of plasminogen showed a similar lytic response as adults. In summary, the reduced fibrinolytic response of OLT fibrin clots to tPA was due to low concentrations of plasminogen and corrected by plasminogen supplementation.

Adult↗
PubMed1995

Perforation during attempted intussusception reduction in children--a comparison of perforation with barium and air.

This paper compares the effects on patients of perforation with barium and with air during attempted intussusception reduction by reviewing the clinical, radiological, surgical and pathological findings and sequelae in seven children who received barium and seven who received air. In both groups perforation occurred in infants under 6 months of age (with one exception) with a long duration of symptoms. All patients with barium enema required resection of bowel whereas only four with air enema required resection. Anesthetic times were longer in those patients with barium perforation in whom the intussusception did not move and there was a large leak. The patients with perforation due to air had a shorter hospital stay with decreased morbidity compared to those with perforation due to barium. Perforation occurred through areas of transmural necrosis in a minority of patients in each group. Perforations through normal bowel and shear injury (with air enema) indicate that increased pressure during the examination is an important factor in some patients. Because perforation with air is so much easier to deal with surgically and the children do better clinically, there is a tendency for some to consider perforation with air an inconsequential situation. However, a potential rare complication with this technique is tension pneumoperitoneum. Keeping this in mind, we continue to use air as the contrast of choice because the procedure in our hands is a safe, quicker and easier technique and we have achieved a substantial improvement in reduction rates. Overall reported perforation rates with air enema compare favourably with those due to barium enema.(ABSTRACT TRUNCATED AT 250 WORDS)

Air↗
PubMed1995

Laparoscopic cholecystectomy versus open cholecystectomy in children: which is better?

Twenty-nine consecutive laparoscopic cholecystectomies (LC) performed between April 1992 and December 1993 were compared with 23 consecutive open cholecystectomies (OC) performed between January 1991 and March 1992 with regard to clinical, surgical, and economic factors. Most patients were Caucasian (> 70%), and symptomatic nonhemolytic cholelithiasis was the most common indication for cholecystectomy. The introduction of LC did not significantly increase the number of cholecystectomies performed per annum. There is a learning curve to LC: the average length of operative time required during the first year was significantly longer than that of OC and the average time for LC during the second year (P < .01). By the second year, the average operative time of LC was not significantly different from OC. There was no conversion from LC to OC, and the complication rate was minor in both groups. The postoperative parenteral analgesic requirement for LC was significantly less than OC (P < .01). The mean length of hospitalization of LC was about three times shorter than that of OC (P < .01). Although the average operating cost per case of LC was significantly more expensive than OC, LC was significantly cheaper because the period of hospitalization was significantly shortened (P < .01). In conclusion, LC is the procedure of choice in the treatment of symptomatic cholelithiasis in children.

Adolescent↗
PubMed1995

Circulating dermatan sulfate and heparan sulfate/heparin proteoglycans in children undergoing liver transplantation.

The liver produces dermatan sulfate (DS), heparan sulfate (HS) and heparin glycosaminoglycans (GAG) and in the presence of hepatic disease, tissue levels of the DS GAG increase dramatically. We hypothesized that in children undergoing liver transplantation plasma levels of DS would be increased. Plasma from children undergoing liver transplantation were tested preoperative, intra operative and post operative at 24-48 h, and 1-3 weeks. Fluctuating levels of DS, HS and heparin anticoagulant activity were detected at all timepoints. The anticoagulant activity was purified and gel chromatography of the material displayed a mean Mr 110,000 D. Reductive elimination decreased the mean Mr 24,000 D indicating the activity resides on a proteoglycan (PG). The purified material was subjected to further chromatography and two peaks of anticoagulant activity resolved, compatible with at least two separate PGs, one with DS GAG chains and the additional PG(s) with HS and heparin GAG chains.

Adolescent↗