Biomedical subjects

K Paya

Publications and source records attributed to K Paya.

21 records · Page 2Linked to original sources

PubMedDate not supplied

[Progressive familial intrahepatic cholestasis (Byler disease): current genetics and therapy].

Progressive familial intrahepatic cholestasis (PFIC) is a congenital liver disease. First symptoms can frequently be seen shortly after birth. Quality and expectation of life are substantially reduced due to severe pruritus and the complications of progressive liver cirrhosis. PFIC is diagnosed on the basis of characteristic clinical and laboratory parameters and genetic analysis after exclusion of other liver diseases leading to intrahepatic cholestasis. Medical therapy is only effective in a proportion of children with PFIC. Partial biliary diversion (PBD) is nowadays considered the therapy of choice in patients with therapy-refractive pruritus. If performed in time, damage to the liver can be delayed or arrested, thus orthotopic liver transplantation (OLT) can be postponed or even avoided in at least some patients with PFIC. Besides providing a current overview of PFIC, we report on three patients who were successfully treated surgically. One patient was subjected to a new technique of PBD (cholecysto-appendicostomy), the other two had OLT.

Appendix↗

PubMedDate not supplied

Open versus laparoscopic appendectomy in children: a comparison of complications.

Although between 4% and 20% of all appendectomies in adults are performed laparoscopically, this procedure is rarely done in the pediatric age group because of the substantially more difficult technique, the expected risks and suspected higher rate of complications. In a prospective study of 500 consecutive appendectomies, we tried to assess the actual rate of complications of each operative approach. We included 362 conventional and 138 laparoscopic appendectomies, the median age of the patients was 10.8 years. The mortality was 0 in both groups. We observed 89 minor and 11 major complications. All major complications (wound infections needing re-operation under general anesthesia, intra-abdominal abscesses, ileus due to adhesions and a case of renal insufficiency because of glomerulonephritis) occurred in the conventional group (n=11, ie, 3% of 362 open appendectomies). Seventy-two minor complications were seen in the same group (20%). In the laparoscopic group, there were 17 (13%) minor complications and no severe complications. This difference was statistically significant. We conclude that in children laparoscopic appendectomy does not carry a greater risk of intra- or postoperative complications and can therefore safely be established as a standard procedure.

Adolescent↗

PubMedDate not supplied

Tissue polypeptide-specific antigen (TPS) in pediatric malignancies.

Tissue Polypeptide Specific Antigen (TPS) may soon be routinely used as a proliferation marker in adult epithelial tumors. So far, no data have been available on normal or pathologic TPS values in children. Therefore the present study was designed to test the marker for the first time in pediatric malignancies. Using a commercial ELISA kit (Beki Diagnostics), serum TPS levels were determined in 270 healthy children and compared with various benign (n = 143) and malignant (n = 58) diseases. In healthy children, we found an age-dependent distribution of TPS values. Median (M) TPS was found to be 105.05 U/l at birth as determined from umbilical cord blood (n = 96). By the end of the first week, the value rose to M = 164 U/l and then continuously decreased with age until reaching the adult level at around 14 years. Patients with benign masses (n = 29) did not show elevated serum TPS in contrast to children with malignancies. Advanced tumor stage, metastases, and low dignity correlated with an increase in serum values. In many cases, chemotherapy and especially surgical resection of the tumor were followed by a decrease of the previously markedly elevated TPS levels. Severe infections and impaired renal function were however related with very high values and this must be taken into account when judging the validity of TPS measurements. The marker appears useful for the differential diagnosis between benign and malignant processes. While previous investigators described its use exclusively in epithelial tumors of adults, we cannot confirm this specifity in pediatric patients: Our data show that elevated TPS values in children can also be observed in nonepithelial tumors.

Adolescent↗