Hye Kyung Chang

Hepatic Fibrosis Scan for Liver Stiffness Score Measurement: A Useful Preendoscopic Screening Test for the Detection of Varices in Postoperative Patients With Biliary Atresia

Objective:Even after successful Kasai portoenterostomy, progressive hepatic fibrosis in postoperative patients with biliary atresia (BA) can be associated with portal hypertension and esophageal or gastric varices. Therefore, early diagnosis and close follow-up of varices are important. We investigated the correlation between the liver stiffness scores measured by FibroScan and the presence of esophageal or gastric varices to examine the usefulness of FibroScan as a preendoscopic screening test for varices. Patients and Methods:A total of 49 of 81 children with BA following successful Kasai operations were enrolled in this study. FibroScan and endoscopic examination were performed prospectively. Results:There were 22 males (44.9%) and the mean age of the patients was 3.8 ± 2.7 years. Esophageal or gastric varices were present in 30 patients (Vx group) and absent in 19 (nVx group). The mean liver stiffness score was significantly higher in the Vx group (21.35 ± 10.31 kPa in the Vx group versus 9.75 ± 8.61 kPa in the nVx group, P < 0.001). The optimal cutoff value of the liver stiffness score for the prediction of a varix was 9.7 kPa with a sensitivity of 0.97 and a specificity of 0.80. Conclusions:Liver stiffness scores measured by FibroScan correlate well with the presence of esophageal or gastric varices. FibroScan is a novel, noninvasive, and useful screening method for the preendoscopic detection of varices in postoperative patients with BA.

Exchange living-donor kidney transplantation: merits and limitations.

Background. The shortage of donor organs is one of the major barriers to transplantation worldwide. After the success of the direct exchange donor (swap) program in Korea since 1991, we have developed a swap-around program. However, reports on the long-term outcomes of exchange donor programs are scarce. Methods. From September 1995 to September 2006, we performed 1193 cases of renal transplantation, including 398 cases from living-unrelated donors. The living-unrelated donors included 129 exchange donors and 269 nonexchange donors. We compared the outcomes of the exchange program with that of the nonexchange program, and examined the merits and limitations of the exchange program. Results. The reasons for the exchange program were ABO incompatibility (n=84, 65.1%), human leukocyte antigen mismatching beyond our criteria (n=39, 30.2%), or positive lymphocyte crossmatch (n=6, 4.7%). The overall 10-year graft survival (86.3%) of exchange transplantation was comparable with that of nonexchange (82.3%) or one- haplotype matched living-related (81.2%) transplantation (P=0.2994). In multivariate analysis, exchange versus nonexchange donors did not affect graft survival. The proportion of blood-type O donors was much lower in the exchange group (29.5%) than in the nonexchange group (42.4%; P=0.026). Blood-type O kidneys were preferentially allocated to blood-type O recipients (78.9%) in the exchange group as compared with the nonexchange group (54.4%; P=0.007). Conclusion. We achieved excellent outcomes by using a donor exchange program as an option to reduce the donor organ shortage. However, the exchange donor program has no added benefit for blood-type O recipients.

Invasive pulmonary aspergillosis after solid organ transplantation: diagnosis and treatment based on 28 years of transplantation experience.

Invasive pulmonary aspergillosis (IPA) is a serious and lethal complication among organ transplant recipients. This report described the clinical manifestations and treatment of IPA over a 28-year period. From January 1979 to December 2007, 3215 organ transplant patients (2954 kidney and 261 liver recipients) were enrolled in the study. Nine patients developed IPA (7 kidney and 2 liver recipients), yielding an incidence of 0.003% (9/3215). Five IPA patients (55.6%) were diagnosed by transbronchial lung biopsy or autopsy, and 3 (33.3%) by sputum culture study. One patient was diagnosed through clinical manifestations and observations of IPA characteristics on chest X ray. We used amphotericin B (n = 4; 44.4%), voriconazole (n = 2; 22.2%), or fluconazole (n = 1; 11.1%) as the primary antifungal agents, but 2 patients could not receive antifungal agents due to rapid disease progression and sequential mortality. This study showed a high mortality rate among IPA patients (55.6%; 5/9). Only patients who received early antifungal agent thereby after a prompt diagnosis recovered from IPA. This survival advantage warrants careful monitoring for invasive fungal infections after organ transplantation with immediate administration of antifungal agents or surgical intervention.

Agranular platelets as a cardinal feature of ARC syndrome.

We aimed to describe abnormal platelet morphology and its clinical significance in infants who were diagnosed with arthrogryposis renal dysfunction and cholestasis (ARC) syndrome. We collected all of the cases of ARC syndrome referred to a single pediatric referral center. In all patients, platelet counts and analysis of platelet morphology were performed with peripheral blood smear specimens. Electron microscopy images were obtained to examine the ultrastructure of the platelets. Over the 12-year period, 12 cases of ARC syndrome were identified. The sex ratio (male:female) was 1:1. The median birth weight was 3.15 kg (range, 2.3 to 3.8 kg). Failure to thrive was observed in all the patients. The major cause of death was recurrent febrile illness and pneumonia. The median age at death was 8.9 months (range, 2.6 to 28.8 kg). Their median body weight at death was 3.1 kg (range, 2.6 to 6.0 kg). Close examination of their peripheral blood smear (n=11) specimens showed large, pale, agranular platelets similar to those seen in gray platelet syndrome. Electron microscopic images of the platelets (n=7) revealed a lack of α; granules. Agranular platelets are a common finding in ARC syndrome. Agranular platelets should be considered as a cardinal feature of ARC syndrome and can be useful as a noninvasive diagnostic marker for ARC syndrome.

Is the Affinity Column–Mediated Immunoassay Method Suitable as an Alternative to the Microparticle Enzyme Immunoassay Method as a Blood Tacrolimus Assay?

BACKGROUND Tacrolimus is a potent immunosuppressive drug used in organ transplantation. Because of its substantial toxic effects, narrow therapeutic index, and interindividual pharmacokinetic variability, therapeutic drug monitoring of whole-blood tacrolimus concentrations has been recommended. We investigated the comparability of the results of 2 immunoassay systems, affinity column-mediated immunoassay (ACMIA) and microparticle enzyme immunoassay (MEIA), comparing differences in the tacrolimus concentrations measured by the 2 methods in relation to the hematologic and biochemical values of hepatic and renal functions. METHODS A total of 154 samples from kidney or liver transplant recipients were subjected to Dimension RxL HM with a tacrolimus Flex reagent cartilage for the ACMIA method and IMx tacrolimus II for the MEIA method. RESULTS Tacrolimus concentrations measured by the ACMIA method (n = 154) closely correlated with those measured by the MEIA method (r = 0.84). The Bland-Altman plot using concentration differences between the 2 methods and the average of the 2 methods showed no specific trends. The tacrolimus levels determined by both the MEIA method and the ACMIA method were not influenced by hematocrit levels, but the difference between the 2 methods (ACMIA - MEIA) tended to be larger in low hematocrit samples (P < .001). CONCLUSION The ACMIA method used for a tacrolimus assay is precise and has advantages, including the lack of a required pretreatment procedure. Furthermore, it is only slightly influenced by the hematologic or biochemical status of the samples.

Home intravenous antibiotic treatment for intractable cholangitis in patients with biliary atresia following Kasai portoenterostomies

Purpose Patients with biliary atresia (BA) treated with Kasai portoenterostomy may later develop intractable cholangitis (IC) that is unresponsive to routine conservative treatment. It may cause biliary cirrhosis and eventually hepatic failure with portal hypertension. Control of IC requires prolonged hospitalization for the administration of intravenous antibiotics. To reduce the hospitalization period, we designed a home intravenous antibiotic treatment (HIVA) which can be administered after initial inpatient treatment. In this study, we reviewed the effects of this treatment. Methods We reviewed medical records of 10 patients treated with HIVA for IC after successful Kasai portoenterostomies performed for BA between July 1997 and June 2009. Results The duration of HIVA ranged from 8 to 39 months (median, 13.5 months). The median length of hospital stay was 5.7 days per month for conventional treatments to manage IC before HIVA and, 1.5 days per month (P = 0.012) after HIVA. The median amount of medical expenses per month was reduced by about one tenth with HIVA. One patient underwent liver transplantation due to uncontrolled esophageal variceal bleeding, but the other nine patients had acceptable hepatic function with native livers. Conclusion HIVA may be an effective primary treatment for IC after Kasai portoenterostomies for BA, and reduce length of hospital stay and medical expense.

Internal jugular vein deformities after central venous catheterisation in neonates: evaluation by Doppler ultrasound.

Aim:  The use of a central venous catheter (CVC) through the internal jugular vein (IJV) in neonates is associated with various complications. We postulated that the risk of vein deformity after removing the CVC is underestimated. This study aimed to evaluate, using Doppler ultrasound, morphological changes in the IJV that had undergone CVC insertion during the neonatal period.

Efficacy of a Negative Conversion Trial and Subsequent Living Donor Kidney Transplant Outcome in Recipients with a Positive Lymphocyte Crossmatch

Background: Patients with a positive lymphocyte crossmatch (LCX) do not undergo kidney transplantation. In such patients, a negative conversion protocol consisting of intravenous immunoglobulin (IVIG), plasmapheresis, and potent immunosuppressant is one of the options for transplantation. Methods: 14 patients who showed a positive LCX with living donors underwent a trial of negative conversion between January 2002 and July 2007. Plasmapheresis was performed every other day, up to 6 times maximum. IVIG was infused after plasmapheresis, with a total dose of 500 mg/kg divided over 6 days. Kidney transplantation was performed immediately after negative conversion. Anti-thymocyte globulin (ATG) or OKT3 induction therapy was used with the combination of tacrolimus, mycophenolate mofetil, and prednisone in the perioperative period. Results: Negative conversion of LCX was achieved in 13 of 14 patients (92.9%). Transplantations were performed successfully in these 13 patients without hyperacute rejection. Four recipients developed acute rejection, which was well controlled by steroid pulse therapy. During the follow-up periods of 45.4 ± 22.0 months, all recipients except 1 showed excellent graft function. Conclusion: Selected patients with a positive LCX can undergo successful transplantation using plasmapheresis, IVIG, and potent immunosuppressants. Recipients with negative conversion of LCX showed acceptable posttransplant results.

Feasibility of a laparoscopic approach for generalized peritonitis from perforated appendicitis in children

Purpose This study evaluated the feasibility of a laparoscopic approach in children with generalized peritonitis secondary to perforated appendicitis. Materials and Methods We retrospectively analyzed the medical records of patients who underwent laparoscopic appendectomy with drainage for generalized peritonitis secondary to perforated appendicitis at our hospital between September 2001 and April 2012. Laparoscopic outcomes were compared with outcomes of an open method for perforated appendicitis. Results Ninety-nine patients underwent laparoscopic appendectomy (LA) for generalized peritonitis from perforated appendicitis, and 87 patients underwent open appendectomy (OA) for perforated appendicitis. Wound infection was more common in the OA group (12.6%) than in the LA group (4.0%; p=0.032). The incidence of intestinal obstruction during long-term follow-up was significantly higher in the OA group (4.6% vs. 0.0% in the LA group; p=0.046). LA was possible in most patients for whom LA was attempted, with a conversion rate of 10.8%. Conversion to OA was affected by the preoperative duration of symptoms and the occurrence of intraoperative complications. Conclusion LA is feasible for use in children with generalized peritonitis from perforated appendicitis, with reasonable open conversion and perioperative complication rates comparable to those of the OA group.

Urodynamic evidence of successful rehabilitation of a severely contracted bladder after renal transplantation

Because of high incidence of postoperative urine leakage, high-grade ureteral reflux and/or obstruction, implantation of the donor ureter into a very small defunctionalized bladder is a major technical difficulty during renal transplantation [1–4]. Despite previous promising results with the implementation of pre-operative augmentation cystoplasty, hydrostatic bladder dilation, and urinary diversion, urodynamic evidence showing the restoration of bladder function has not yet been reported. Herein, we report with urodynamic evidence the successful rehabilitation of a severely contracted small bladder of <30 cm capacity after renal transplantation using a stented ureteroureterostomy. A 42-year-old female patient who has been dependent on maintenance dialysis for 16 years received a renal allograft from a younger brother. During the pre-operative urodynamic evaluation, patient complained of low abdominal discomfort at 26 cm filling and urinary leakage was observed. Bladder margins were smooth on the cystogram (Fig. 1a). Voiding was possible by abdominal straining only at a maximal cystometric capacity of 36 cm. A grade I vesicoureteral reflux into the left native ureter was observed during the voiding phase. Concomitant uroflowmetry revealed a tower shape curve with a maximal flow rate (Qmax) of 16.1 ml/s. The graft was implanted in the right iliac fossa as usual. The bladder was fibrotic and contracted. Because it has been reported that the native ureter may be safely ligated in patients with anuria or oliguria [5], we performed ureteroureterostomy between the donor ureter and distal native ureter over a double-J stent after ligation of the proximal native ureter. Immunosuppression was maintained with cyclosporine micro-emulsion and low-dose steroids. Starting from postoperative 14th day, indwelling urinary catheter was clamped until the patient developed an urge to void and then released. At postoperative 19th day, a voiding cystogram (Fig. 1b) was performed and the stent was removed after confirming the bladder volume to be more than 130 cm. Urinary catheter was removed the next day. Postoperative ultrasonogram showed no abnormal findings. There was no acute rejection or surgical complication. After 2-months of transplantation, the patient complained of daytime frequency, urgency and urgency incontinence, nocturia, and stress urinary incontinence. Voiding diary revealed polyuria with a functional bladder capacity of 300 cm. Detrusor pressure at voiding was 36 cm water column, after 279 cm filling. Previously noted daytime frequency, urgency and urge incontinence and left vesicoureteral reflux were diminished after 3 months of transplantation. Although much improved, nocturia (more than two times per night) because of polyuria and stress urinary incontinence remained. The functional bladder capacity was now increased to 420 cm. Maximal cystometric capacity was checked to be 440 cm and detrusor pressure at voiding was 44 cm water column. Concomitant uroflowmetry revealed a bellshaped curve with Qmax 73.2 ml/s and residual volume of 30 cm. After 14 months of transplantation, urinary symptoms, voided diary, and uroflowmetry findings remained unchanged (Fig. 2). After bladder augmentation or diversion, renal recipients having contracted bladder are known to be at increased risk for morbidities and complications [1–4], and these can lead to reluctance in undergoing renal transplantation in these patients. The use of the native defunctionalized bladder, if possible, should not be overlooked. In 1974, Tanagho [6] reported that defunctionalization of a normal bladder does not limit its ability to recover its function. Serrano et al. [7] reported that continuous bladder cycling before transplantation remains the best way to rehabilitate, artificially, the prolonged defunctionalized bladder. Salvatierra et al. [8] introduced the intravesical implantation of the transplant ureter into the small, defunctionalized bladder. However, most of the proposed techniques are time-consuming, complex and show increased morbidity and surgical complications. Our stented ureteroureterostomy is more physiologic, technically convenient and appears to guarantee success against both ureteral reflux and obstruction. Moreover, both bladder capacity and contractility returned to a normal state as early as 2 months after transplantation. These results suggest that it may be unnecessary for recipient with small and defunctionalized bladder to pre-operatively perform the procedures