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Biomedical subjects

P Friend

Publications and source records attributed to P Friend.

At least 19 recordsLinked to original sources

Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.

UNLABELLED: One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. IN CONCLUSION: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.

Arteriovenous Fistula

Glomerulonephritis with end-stage liver disease in childhood.

Renal biopsies were done perioperatively in 18 children receiving liver grafts. All specimens showed glomerulonephritis, which was mesangial-proliferative in 15 and mesangio-capillary in 3. Of the 16 children who were alive four or more months after transplantation, only 1 showed progressive deterioration of renal function; 1 other had a subnormal but static glomerular filtration rate. In all 6 children who had proteinuria before operation, the urine became normal.

Adolescent

Liver transplantation in 100 children: Cambridge and King's College Hospital series.

OBJECTIVE: To review the results of the Addenbrooke's and King's College Hospital children's liver transplantation programme. DESIGN: Retrospective analysis of the first 100 children to receive liver grafts at Addenbrooke's Hospital, Cambridge, from December 1983 to March 1990. SETTING: Addenbrooke's Hospital, Cambridge, and King's College Hospital, London. SUBJECTS: 153 children assessed for liver transplantation, of whom 22 died before a donor became available and 31 were considered unsuitable. 100 children received grafts, of whom 27 had second grafts. RESULTS: One year actuarial patient survival was 71%, with 57% one year graft survival. In the last two years survival rates had improved considerably, with 86% of patients and 63% of grafts surviving for at least one year. Sixty five children were alive 12 to 86 months after transplantation; 63 were well and leading normal active lives and 56 had entirely normal liver function. Children's growth and development were essentially normal, with many showing remarkable catch up growth. CONCLUSIONS: Liver transplantation offers children with terminal liver disease a high chance of a return to full quality life and normal development. Improved surgical and medical care have progressively improved survival. The timing of transplantation is critical but has been constrained particularly by the availability of donors and resources.

Adolescent

The improved lytic function and in vivo efficacy of monovalent monoclonal CD3 antibodies.

A series of hybrid-hybridomas were derived by the cell fusion of a CD3 antibody-secreting hybridoma with other Ig-producing cell lines. The Ig molecules secreted by these hybrid-hybridomas were fractionated by ion-exchange chromatography, and fractions containing monovalent CD3 antibodies were tested for complement-mediated lysis of T cells. Two monovalent CD3 antibodies with mixed heavy chain isotypes were very poor in lysis but, in contrast, a monovalent antibody possessing two identical rat gamma 2b heavy chains but two non-identical light chains was found to be more lytic with human complement than the parental bivalent CD3 antibody. The difference in lysis was not readily explicable in terms of a difference in complement activation at the level of C1q binding or cell-associated C3. A highly purified batch of this lytic monovalent CD3 antibody was prepared in a form suitable for in vivo therapy. In a preliminary clinical study in one patient with a T lymphoma in leukemic phase this monovalent CD3 antibody was found to be very effective in depleting CD3+ tumor cells in the peripheral blood and bone marrow. The cells in the peripheral blood were completely cleared and there was no evidence for antigenic modulation. In addition the antibody was able to reverse cell-mediated kidney rejection in three kidney graft patients. These results suggest that monovalent antibody may be effective in vivo as well as in vitro and that a fuller clinical evaluation is justified.

Adolescent

Ventral marking in the male Mongolian gerbil reflects present and future reproductive investments.

Ventral scent-marking in the male Mongolian gerbil, Meriones unguiculatus, may be related to reproductive potential. In four experiments involving nine groups, males' marking during 5-min. open field tests correlated with their social and reproductive status. The lowest levels of marking occurred among individually housed or subordinate males of a pair, followed by males exposed to ovariectomized females, and dominant males of a pair. Higher levels of marking occurred with vasectomized males living with intact females or following the removal of males from a nuclear family. The most frequent marking occurred when males were housed with pregnant females and with females and their offspring. The proximate stimuli leading to high marking appear to be the sexual availability of reproductively competent females.

Animals

Influence of dietary restriction on immunologic function and renal disease in (NZB x NZW) F1 mice.

In (NZB x NZW)F(1) (B/W) mice, moderate caloric intake [10 kcal (41.8 kJ) per day] from the time of weaning was associated with maintenance of lower body weight, greater capacity of spleen cells to be stimulated with T-cell mitogens, and better preserved capacity to generate cytotoxic cells in response to in vitro and in vivo stimulation with allogeneic tumor cells. Plaque-forming cell response to sheep erythrocytes was also well maintained in animals on the restricted diets when sensitization was accomplished either in vitro or in vivo. Spontaneous suppressor cell activity against plaque-forming cells that developed in controls did not appear in the mice on the restricted diet. Significantly less circulating antibody to native DNA was present in the blood of mice 10 months of age when their dietary intake had been restricted. Histological analysis revealed that the development of renal disease and the deposition of gamma globulin in the glomerular capillaries was markedly inhibited in the mice on restricted diets. Dietary restriction from the time of weaning thus appears to prolong significantly the life of autoimmunity-prone (NZB x NZW)F(1) male and female mice and to alter lymphoid cell immune function, thereby decreasing the autoimmune processes and immunological assault associated with progressive renal disease in these animals.

Animals

Nursing 1974--76. 2.

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Comprehensive Health Care

Nursing 1974--76. 1.

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Child Health Services

Deficiency of the second component of complement (C2) with chronic vasculitis.

A patient had complete deficiency of the second component of complement associated with chronic vasculitis and increased susceptibility to infection. We discuss here results of the complement profile, histocompatibility typing, and studies of the functional properties of patient plasma or serum in chemotaxis and opsonization in relation to the disease entity and host susceptibility to infection.

Adult