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Biomedical subjects

R Superina

Publications and source records attributed to R Superina.

29 records · Page 2Linked to original sources

Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies.

Congenital absence of the portal vein (CAPV) is an unusual condition that often is associated with other anomalies. This is the first report of reduced-size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no portal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complicated by severe mesenteric edema caused by an increase in portal pressure when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indicate that the CAPV can be classified into one of two groups of portasystemic anomalies.

Abnormalities, Multiple↗

Post-transplant recurrent hepatitis B viral liver disease. Viral-burden, steatoviral, and fibroviral hepatitis B.

Recurrence of hepatitis is a well-documented complication of hepatitis B liver disease, post-transplantation. It is well established also that the earliest hepatocellular change is the appearance of hepatitis B viral (HBV) markers and that the disease is rapidly progressive. In this article on 17 liver transplants in 16 HBV positive patients with long-term follow-ups (100-1234 days), the distinctive pathologic features of this disease are emphasized: the extreme viral load, the steatosis, and/or fibrosis. An attempt to quantitate the magnitude of the viral burden was made and the result was a staggering figure. In one patient, an estimated 10(18) HBV core particles were present in the liver. One of two patterns of progression were noted. In four patients in addition to the massive nuclear hepatitis B core antigen (HBcAg) and cytoplasmic hepatitis B surface antigen (HBsAg) positivity, superimposed hepatitic changes led to diffuse hepatic fibrosis (fibroviral hepatitis B); and in another six patients, extraordinary hepatocellular viral marker positivity and steatosis were the hallmarks (steatoviral hepatitis B). Steatosis is not usually considered a feature of HBV liver pathology. These results suggest that more than one type of posttransfusion recurrent hepatitis B liver disease exists pathologically.

Fatty Liver↗

Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features.

Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features.

Cystic Adenomatoid Malformation of Lung, Congenita↗

Cytomegalovirus infections in pediatric liver transplantation.

From 1986 to 1989, 26 consecutive pediatric liver transplant recipients were followed up at The Hospital for Sick Children, Toronto, Canada. The patients were reviewed to assess the incidence of infection with cytomegalovirus, the severity of disease, and the relationship of recipient and donor serostatus to cytomegalovirus disease. Overall, the incidence of infection was 54% (14/26). Over 90% of patients who were seropositive or whose donors were seropositive developed evidence of cytomegalovirus infection after transplantation. Forty-three percent (6/14) of those with cytomegalovirus infections developed severe, fatal cytomegalovirus disease, despite treatment with immunoglobulins and ganciclovir (Syntex Laboratories Inc, Palo Alto, Calif) or foscarnet sodium (Astra, Pharmaceutical Products Inc, Westborough, Mass). Of all posttransplant deaths, two thirds were associated with severe cytomegalovirus infection. Cytomegalovirus-related deaths occurred in three of four seronegative patients with seropositive donors and three of six seropositive patients with seropositive donors. Therefore, a seropositive donor appeared to be a major risk factor for severe cytomegalovirus disease.

Adolescent↗

Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurologically normal children.

Antireflux surgery was performed in 234 children over a 5-year period; 153 were neurologically impaired (NI) and 81 were neurologically normal (NN). Initial presentation, demographic data, and type of antireflux operation were similar in the two groups. Eighty-six percent of the NI group versus 30% of the NN group had gastrostomy tubes placed. The incidence of late postoperative complications was 26% in the NI group and 12% in the NN group (P less than .01). During the late postoperative period, NI children underwent reoperation four times as frequently as NN children (19% v 5%, respectively; P less than .01). Wrap herniation accounted for 38% of complications and 59% of reoperations in the late postoperative period. Mortality due to aspiration occurred in 9% of the NI group versus 1% of the NN group. Combined failure rate (reoperation plus aspiration-induced deaths) was 28% in NI and 6% in NN (P less than .01). We conclude that neurological status is the major predictor of operative success and that wrap herniation due to crural disruption is the most common cause of operative failure.

Adolescent↗

Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report of two cases.

The combined anomalies of pulmonary agenesis with esophageal atresia and distal tracheoesopahgeal fistula are an exceedingly rare and highly lethal association. The longest survivor in the literature is 10 months. We describe two neonates with this association who underwent primary repair shortly after birth and who are alive and well at 24 and 18 months. One of these neonates also demonstrated duodenal atresia. The prime goal in the management of these patients is early protection and preservation of respiratory units. This goal is best accomplished by primary repair of the tracheoesophageal lesion, when feasible, rather than by more complex procedures.

Abnormalities, Multiple↗

Ischemic bowel after primary closure for gastroschisis.

Between 1983 and 1986, four newborns who had primary closure of gastroschisis had postoperative ischemic bowel. Suspicion was raised almost immediately after closure that something was wrong inside the abdomen when there was persistent acidosis, sepsis, abdominal wall redness, and a generalized worsening condition. All four neonates were re-explored. Necrotic bowel was found, and three required silon pouch closure. The two survivors were left with a temporary short gut. Whether the cause of the bowel ischemia in the four babies was due to excessive intraabdominal pressure, volvulus, or the intestines being too vigorously manipulated, is speculative. Therefore, excessive manipulation and compression of gastroschisis contents seem unwise; if such a newborn has persistence of the above signs and symptoms, immediate reoperation and decompression are warranted.

Abdominal Muscles↗

Lymphocyte function in anergic patients.

The lymphocyte function of anergic surgical patients who are at increased risk for sepsis and mortality was studied. In vitro lymphocyte responses appear to be normal in most instances, in that over 80% of patients showed a normal response in a standardized mixed leucocyte culture reaction. Similarly, 56% of the lymphocytes from anergic patients showed a positive in vitro proliferative response with PPD. The ability of in vitro-activated lymphocytes to elicit a skin reaction was determined by culturing the cells of anergic patients with PPD and then reinjecting the lymphocytes or their supernatants intradermally into the original donor. When there was a positive proliferative response to PPD in vitro, the reinjected cells or supernatant elicited a positive skin reaction in 79% of the anergic patients. In contrast, a skin reaction was obtained in less than 20% of the instances when there was no in vitro proliferation to PPD or when the cells were cultured without antigen. These results suggest that one of the acquired immune defects in these anergic patients is an in vivo block of lymphocyte activation.

Cells, Cultured↗