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Cholestyramine therapy in patients with a paucity of intrahepatic bile ducts
434
INTERNATIONAL
Vitamin K dependent hypoprothrombinemia with a prolonged clotting time and a deficiency of factors II, IX and X were present in all infants with hematoperitoneum. This deficiency which is present in approximately 30 per cent of all newborns tested, together with trauma to a large fixed organ were the two major contributing causes of this lesion, The regime of management presently being utilized includes: resuscitative measures (incubator, O,, #8 French umbilical vein catheter, fresh 0 Rh negative blood to overcome shock, vitamin K 10 mg. iv. and 10 mg. I.M., fresh frozen plasma 5 ml/lb. after a hemoglobin of 10 Gm./lOO ml. had been reached then additional fresh blood until the hemoglobin reaches 14, Na-bicarbonate I.V. to correct the acidosis, antibiotics, gamma globulin, and calcium gluconate and fluids as 10 per cent and 5 per cent dextrose); observation (careful monitoring of vital signs and abdominal girth, x-rays of chest and abdomen, urine output, repeat the prothrombin index every 2 hours until a level of 60 per cent or higher is obtained, possible deficiency to be evaluated), then laparotomy (evacuation and measurement of the blood, obtain hemostasis with the cautery, topical thrombin and gelatin sponge, and drainage).-Howard Cooperman.
CONGENITALHEPATIC FIBROW AS A CAUSE OF PORTAL HYPERTENSION: REPORT OF Two CASES. R. E. Hermunn and W. A. Hawk. Surgery 62: 1095-1099, December 1967. Congenital hepatic fibrosis was described in 1961 as a cause of portal hypertension in children and young adults. Two cases are presented. The first case, a 14-year old girl, was treated with an end-to-side portacaval shunt. The second patient, a 16-year old girl, was treated by splenectomy because splenomegaly was the only complication observed. Liver biopsies from each case confirmed the diagnosis. In this condition the splenoportogram is the essential diagnostic study and will show a patent portal vein without evidence of extrahepatic obstruction. Liver function is almost always normal, and the prognosis for life should be excellent once protection is provided against variceal hemorrhage. The importance of differentiating this lesion from acquired cirrhosis is emphasized.-Daniel T. Cloud. CHOLESTYHAMNE THERAPY IN PATIENTS WITH A PAUCITY OF INTRAHEPATIC BILE DUCTS. Haruey L. Sharp, James 3. Carey, Jr., James G. White, and William Kriuit. J. Pediat. 71:723-736, November 1967.
ABSTRACTS OF PEDIATRIC SURGERY
These authors summarize their observations on 5 children with paucity of bile ducts in the portal triads before and after the administration of cholestyramine, a bile acid binding resin. All patients were demonstrated to have an unobstructed extrahepatic biliary system at laparotomy and/or cholangiograms. Cholestyramine was administered in th e powder form with fruit juices. The patients received a total daily amount varying between 6 and 24 Gm. As a result of this therapy not only was pruritus relieved but normal serum concentrations of bile acids have been attained in two of the children. Concomitantly there has been associated improvement in liver function and physical growth in these 2 patients. The third and most severely affected child is currently responding in a similar manner. -George W. Holcomb, jr. GENITOURINARY
TRACT
KIDNEY INJURIES IN CHILDREN. 2’. S. Morse, 1. P. Smith, W. H. R. Howard and M. 1. Rowe. J. Urol. 98:539-547, November 1967. A report of 80 cases of renal trauma from the Children’s Hospital, Columbus, Ohio. Eighteen cases required operation. Two were shattered kidneys requiring nephrectomy; 3 were giant hydronephrotic kidneys, best removed; I was a nephroblastoma, which was removed; 2 had early exploration, hemorrhage could not be controlled and nephrectomy was necessary. The authors felt these two kidneys might have been salvaged by waiting a little longer before exploration. Three cases with extravasation were followed to a point where when eventually explored, nephrectomy was abscess was prerequired in 2, and a perinephric sent in a third. In 7 cases with extravasation where a semielective operation was employed 2 to 7 days after injury, it was possible to do a reparative operation in each, and the long-term follow-up has been satisfactory. The authors stress that every child suspected of renal injury should have an excretory urogram, preferably an infusion pyelogram. If this does not give adequate information, retrograde pyelography should be performed. Most of their patients requiring this second study were found to have a clear indication for operation. Eight of 32 children with left-sided kidney injury had also rupture of the spleen; 40 per cent of the overall group of patients had at least one other injury in addition to the kidney. Renal contusions can be treated conservatively. Most children who need operation can be treated
INTERNATIONAL
Vitamin K dependent hypoprothrombinemia with a prolonged clotting time and a deficiency of factors II, IX and X were present in all infants with hematoperitoneum. This deficiency which is present in approximately 30 per cent of all newborns tested, together with trauma to a large fixed organ were the two major contributing causes of this lesion, The regime of management presently being utilized includes: resuscitative measures (incubator, O,, #8 French umbilical vein catheter, fresh 0 Rh negative blood to overcome shock, vitamin K 10 mg. iv. and 10 mg. I.M., fresh frozen plasma 5 ml/lb. after a hemoglobin of 10 Gm./lOO ml. had been reached then additional fresh blood until the hemoglobin reaches 14, Na-bicarbonate I.V. to correct the acidosis, antibiotics, gamma globulin, and calcium gluconate and fluids as 10 per cent and 5 per cent dextrose); observation (careful monitoring of vital signs and abdominal girth, x-rays of chest and abdomen, urine output, repeat the prothrombin index every 2 hours until a level of 60 per cent or higher is obtained, possible deficiency to be evaluated), then laparotomy (evacuation and measurement of the blood, obtain hemostasis with the cautery, topical thrombin and gelatin sponge, and drainage).-Howard Cooperman.
CONGENITALHEPATIC FIBROW AS A CAUSE OF PORTAL HYPERTENSION: REPORT OF Two CASES. R. E. Hermunn and W. A. Hawk. Surgery 62: 1095-1099, December 1967. Congenital hepatic fibrosis was described in 1961 as a cause of portal hypertension in children and young adults. Two cases are presented. The first case, a 14-year old girl, was treated with an end-to-side portacaval shunt. The second patient, a 16-year old girl, was treated by splenectomy because splenomegaly was the only complication observed. Liver biopsies from each case confirmed the diagnosis. In this condition the splenoportogram is the essential diagnostic study and will show a patent portal vein without evidence of extrahepatic obstruction. Liver function is almost always normal, and the prognosis for life should be excellent once protection is provided against variceal hemorrhage. The importance of differentiating this lesion from acquired cirrhosis is emphasized.-Daniel T. Cloud. CHOLESTYHAMNE THERAPY IN PATIENTS WITH A PAUCITY OF INTRAHEPATIC BILE DUCTS. Haruey L. Sharp, James 3. Carey, Jr., James G. White, and William Kriuit. J. Pediat. 71:723-736, November 1967.
ABSTRACTS OF PEDIATRIC SURGERY
These authors summarize their observations on 5 children with paucity of bile ducts in the portal triads before and after the administration of cholestyramine, a bile acid binding resin. All patients were demonstrated to have an unobstructed extrahepatic biliary system at laparotomy and/or cholangiograms. Cholestyramine was administered in th e powder form with fruit juices. The patients received a total daily amount varying between 6 and 24 Gm. As a result of this therapy not only was pruritus relieved but normal serum concentrations of bile acids have been attained in two of the children. Concomitantly there has been associated improvement in liver function and physical growth in these 2 patients. The third and most severely affected child is currently responding in a similar manner. -George W. Holcomb, jr. GENITOURINARY
TRACT
KIDNEY INJURIES IN CHILDREN. 2’. S. Morse, 1. P. Smith, W. H. R. Howard and M. 1. Rowe. J. Urol. 98:539-547, November 1967. A report of 80 cases of renal trauma from the Children’s Hospital, Columbus, Ohio. Eighteen cases required operation. Two were shattered kidneys requiring nephrectomy; 3 were giant hydronephrotic kidneys, best removed; I was a nephroblastoma, which was removed; 2 had early exploration, hemorrhage could not be controlled and nephrectomy was necessary. The authors felt these two kidneys might have been salvaged by waiting a little longer before exploration. Three cases with extravasation were followed to a point where when eventually explored, nephrectomy was abscess was prerequired in 2, and a perinephric sent in a third. In 7 cases with extravasation where a semielective operation was employed 2 to 7 days after injury, it was possible to do a reparative operation in each, and the long-term follow-up has been satisfactory. The authors stress that every child suspected of renal injury should have an excretory urogram, preferably an infusion pyelogram. If this does not give adequate information, retrograde pyelography should be performed. Most of their patients requiring this second study were found to have a clear indication for operation. Eight of 32 children with left-sided kidney injury had also rupture of the spleen; 40 per cent of the overall group of patients had at least one other injury in addition to the kidney. Renal contusions can be treated conservatively. Most children who need operation can be treated