Secondary abnormality of carnitine biosynthesis results from carnitine reabsorptional system defect in juvenile visceral steatosis mice

We characterized the L-carnitine transport system which is defective in the kidney of juvenile visceral steatosis (JVS) mice by using kidney slices and carnitine-related compounds, and evaluated the influence of the transport defect on the biosynthetic pathway of carnitine. The JVS mouse transport system defect, calculated as the difference in the transport activity between control and JVS mice, was simulated in control by gamma-butyrobetaine (gamma-BB) and acetyl L-carnitine. gamma-BB hydroxylase activity in the liver of JVS mice was double that of control mice, but the hepatic level of gamma-BB in JVS mice was lower than in control mice, suggesting that the conversion of gamma-BB to carnitine is not activated in the liver of JVS mice. JVS mice showed higher fractional excretions not only of L-carnitine but also of gamma-BB and acetyl L-carnitine than control mice, indicating disturbed reabsorption of gamma-BB and acetyl L-carnitine. The disturbed reabsorption of gamma-BB in JVS mice is consistent with the fact that the amount of urinary gamma-BB in JVS mice was four times that of control. The sum of the concentrations of L-carnitine, acetyl L-carnitine and gamma-BB in the urine of JVS mice was not significantly different from that of the control, suggesting no remarkable increase of biosynthesis of gamma-BB and carnitine in JVS mice. All these findings suggest that the carnitine transport system plays a role in the transport of gamma-BB and that carnitine deficiency is aggravated by the disturbed reabsorption of gamma-BB in the kidney.     

Scrotal ulcer occurring in patients with acute promyelocytic leukemia during treatment with all-trans retinoic acid

STUDY OBJECTIVE: To evaluate the efficacy of conservative laparoscopic surgery in a series of patients with stage III-IV endometriosis. DESIGN: Prospective study (Canadian Task Force classification II-1). SETTING: University-affiliated hospital. PATIENTS: All 141 women who underwent conservative operative laparoscopy for stage III-IV endometriosis between January 1993 and December 1996 and were followed for a minimum of 6 months. INTERVENTIONS: Laparoscopic procedures performed with scissors, bipolar coagulation, and hydrodissection. MEASUREMENTS AND MAIN RESULTS: Clinical examination, transvaginal ultrasonography, and pain questionnaire were scheduled every 6 months postoperatively. The cumulative proportion of pregnant patients and cumulative recurrence rate were calculated by Kaplan-Meier method. Twenty-five women (44%) with infertility became pregnant. Twenty-three (51%) had stage III and two (16.7%, p <0.05) had stage IV endometriosis. The 24-month cumulative pregnancy rate was 57.5%. Thirty-one women (22%) reported pain recurrence during follow-up. Five (3.5%) recurrences were confirmed by histologic examination and eight (5.7%) were documented only by clinical and ultrasonographic findings. No recurrence occurred in the first 6 months of follow-up. CONCLUSION: Operative laparoscopy seems to be effective treatment for stage III endometriosis. A larger series with longer follow-up is necessary to clarify its role in the management of stage IV disease. (J Am Assoc Gynecol Laparosc 6(1):55-58, 1999)     

Complete neurological recovery of an adult patient with type II citrullinemia after living related partial liver transplantation

Type II citrullinemia is an adult-onset hepatocerebral disease caused by a deficiency of argininosuccinate synthetase in liver. A 25-year-old Japanese man suddenly developed encephalopathy, showing disorientation and flapping tremor. Plasma concentrations of ammonia and citrulline were extremely high, and hepatic argininosuccinate synthetase activity was deficient. The patient's condition deteriorated rapidly in spite of intensive medications. Therefore, we performed a partial liver transplantation using a graft obtained from his healthy 61-year-old father. After surgery, his neurological symptoms soon disappeared and plasma levels of ammonia and citrulline were normalized within 3 months after operation. Type II citrullinemia is one fulminant form of various liver-based metabolic diseases, and immediate liver transplantation is necessary to rescue patients with this disease. As liver transplantation from cadaveric donor is still not possible in Japan, it seems justifiable to use living related partial liver transplantation for our patient.     

Synthesis of 2-alkyloyl-1,4,3,6-dianhydrosorbitol-5-sulfates and evaluation of the surface active properties

New anionic surfactants 2-alkyloyl-1,4,3,6-dianhydrosorbitol-5-sulfates were synthesized by sulfation of 2-alkyloyl-1,4,3,6-dianhydrosorbitol. Studies on the surface active properties such as surface tension, dispersibility and emulsifiability of these surfactants were carried out. The results showed these surfactants are better dispersants and emulsifiers than Span 80 and Span 85.     

Successful treatment of severe cytomegalovirus retinitis with foscarnet and intraocular injection of ganciclovir in a myelosuppressed unrelated bone marrow transplant patient

PURPOSE: To compare U.S. medical student Match results in 1996 for 19 categorical residency positions by specialty with those of the overall Match reported by the National Resident Matching Program (NRMP). METHOD: Data for the numbers of "active" senior U.S. student applicants (those who submitted rank lists), the numbers of U.S. seniors matched, and the numbers of unfilled positions for 19 specialties were obtained from a variety of sources. Chi-square analysis was performed to compare Match results for each independent specialty with the overall Match results. The level for statistical significant was set at p < .005. RESULTS: Eight specialties were identified as significantly more competitive than the overall Match process for both the percentage of U.S. seniors who successfully matched in that specialty and the ratio of unmatched U.S. senior applicants to unfilled categorical positions. Five specialties were identified as significantly less competitive for these two measures. Six specialties showed no significant difference in the percentages of U.S. students matching, but for three of these specialties there were more unmatched students than unfilled categorical positions. CONCLUSION: U.S. medical student Match results for categorical residency positions for different specialties vary significantly from the overall Match process. This information can be used in counseling senior medical students on their specialty selection and the residency application process.     

Pancreatic secretory trypsin inhibitor as a diagnostic marker for adult-onset type II citrullinemia

Adult-onset type II citrullinemia is characterized by which deficiency of argininosuccinate synthetase (ASS) protein is found specifically in the liver of patients. Our recent study using differential messenger RNA (mRNA) display showed that the expression of human pancreatic secretory trypsin inhibitor (hPSTI) mRNA increases significantly in the liver of all type II patients tested. In the present work, we found that the concentration of hPSTI protein was higher in the liver of type II patients than controls. Because it is well known that PSTI is a secretory protein and because serum PSTI has been proposed as a marker of pancreatic and nonpancreatic diseases, we measured the hPSTI level in the blood of type II patients and compared it with other serum markers such as elastase 1, trypsin, phospholipase A2, alpha-fetoprotein, CA19-9, and C-reactive protein (CRP). We found a significant increase in serum hPSTI level with no change in the other serum markers. These results suggest that serum hPSTI is useful as a diagnostic marker for adult-onset type II citrullinemia.