The Outcome of Childhood Immunoglobulin A Nephropathy with Acute Kidney Injury at the Onset of the Disease-National Study.

Introduction: IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. Decreased glomerular filtration rate is a known risk factor for disease progression. Aim: We aimed to examine factors that may contribute to disease progression in children that present with impaired eGFR at the onset of IgAN. Materials and methods: Of the 175 patients with IgAN from the Polish Registry of Children with IgAN and IgAVN, 54 (31%) patients with IgAN who had an onset of renal function impairment (GFR < 90 mL/min) were eligible for the study. All of them were analyzed for initial symptoms (GFR according to Schwartz formula, creatinine, proteinuria, IgA, C3), renal biopsy result with assessment by Oxford classification, treatment used (R-renoprotection, P-prednisone+R, Aza-azathioprine+P+R, Cyc-cyclophosphamide+P+R, CsA-cyclosporine+P+R, MMF-mycophenolate mofetil+P+R), and distant follow-up. Based on the GFR score obtained at the end, patients were divided into two groups: A-GFR > 90 mL/min and B-GFR < 90 mL/min. Results: In the study group, the mean age of onset was 12.87 ± 3.57 years, GFR was 66.1 ± 17.3 mL/min, and proteinuria was 18.1 (0-967) mg/kg/d. Renal biopsy was performed 0.2 (0-7) years after the onset of the disease, and MESTC score averaged 2.57 ± 1.6. Treatment was R only in 39% of children, P+R in 20%, Aza+P+R in 28%, Cyc+P+R in 9%, CsA+P+R in 7%, and MMF+P+R in 3%. The length of the observation period was 2.16 (0.05-11) years. At the follow-up, Group A had 30 patients (56%) and Group B had 24 patients (44%). There were no significant differences in any of the other biochemical parameters (except creatinine) or proteinuria values between the groups and the frequency of the MESTC score ≥ 2 and <2 was not significantly different between Groups A and B. Patients with normal GFR at the follow-up (Group A) were significantly more likely to have received prednisone and/or immunosuppressive treatment than those in Group B (p < 0.05) Conclusions: In a population of Polish children with IgAN and decreased renal function at the onset of the disease, 56% had normal GFR in remote observation. The use of immunosuppressive/corticosteroids treatment in children with IgAN and impaired glomerular filtration rate at the beginning of the disease may contribute to the normalization of GFR in the outcome, although this requires confirmation in a larger group of pediatric patients.

Is Elective Status a Predictor of Poor Survival in Liver Retransplantation?

BACKGROUND: Orthotopic liver retransplantation (reLT) is considered to have poorer outcomes than primary transplantation. The objective of this study was to analyze the impact of medical urgency status as a predictor of patient survival after reLT. METHODS: Forty-nine patients who underwent reLT were included in this retrospective study. Urgent or elective status was based on the judgment of the surgical team, selected variables, and the Model for End-Stage Liver Disease score. Multivariate analysis was performed to identify variables associated with patient survival following reLT. RESULTS: Overall survival of the patient cohort was 57% at 1 year and 54.3% at 3 years after reTL. Survival in urgent-status patients was 68.8% and 63.4% at 1 and 3 years, respectively, whereas the survival rate for elective patients was 40.0% at both time points. Mortality was significantly associated with elective status (hazard ratio [HR], 2.42; P = .046) at 1 year, but was no longer significant (HR, 2.19; P < .069) after 3 years of follow-up. CONCLUSIONS: Elective status is associated with poorer outcome. Patient selection determines long-term survival more than any other single factor, so for patients designated to an elective status, prompt retransplantation should be encouraged.

IgA Nephropathy in Children: A Multicenter Study in Poland.

IgA nephropathy (IgAN) is the most common form of glomerulonephritis in pediatric population. The clinical presentation of the disease in children ranges from microscopic hematuria to end-stage kidney disease. The aim of the study was to retrospectively assess clinical and kidney biopsy features in children with IgAN. We assessed a cohort of 140 children, 88 boys, 52 girls with the diagnosis of IgAN in the period of 2000-2015, entered into the national Polish pediatric IgAN registry. The assessment included the following: proteinuria, hematuria, glomerular filtration rate (GFR), arterial blood pressure, and the renal pathological changes according to the Oxford classification and crescents formation, as modifiable and unmodifiable risk factors. The incidence of IgAN in Poland was set at 9.3 new cases per year. The mean age at onset of IgAN was 11.9 ± 4.3 years, and the most common presentation of the disease was the nephritic syndrome, recognized in 52 % of patients. Kidney biopsy was performed, on average, 1.3 ± 2.0 years after onset of disease. Based on the ROC analysis, a cut-off age at onset of disease for GFR <90 mL/min/1.73 m2 (risk factor of progression) was calculated as 13.9 years. Unmodifiable lesions: segmental sclerosis, tubular atrophy/interstitial fibrosis (S1, T1-2) in the Oxford classification and crescents in kidney biopsy were significantly more common in Gr 1 (>13.9 years) compared with Gr 2 (<13.9 years), despite a significantly shorter time to kidney biopsy in the former. We conclude that IgAN in children may be an insidious disease. A regular urine analysis, especially after respiratory tract infections, seems the best way for an early detection of the disease.

Uncommon branching pattern of the hepatic arteries in a living donor: a case report and brief literature review.

Numerous variations of the hepatic arteries are common in surgical patients. We present a 35-year-old woman who was admitted to our department in order to assess possibility of becoming living donor. Preoperative computed tomography scan revealed anomalous branching pattern of the hepatic arteries. In this case right posterior sectoral artery has been given off by the greater pancreatic artery, left hepatic artery has been replaced by the artery arising from the left gastric artery and double segment 4 branches have been observed. To the best of our knowledge, this pattern has not been described in the literature, yet.

Increased Serum IgA in Children with IgA Nephropathy, Severity of Kidney Biopsy Findings and Long-Term Outcomes.

The aim of the study was to determine whether an elevated IgA level at the time of the diagnosis of IgA nephropathy has an effect on the severity of kidney biopsy findings and long-term outcomes in children. We retrospectively studied 89 children with IgA nephropathy who were stratified into Group 1- elevated serum IgA and Group 2 - normal serum IgA at baseline. The level of IgA, proteinuria, hematuria, glomerular filtration rate (GFR) and hypertension (HTN) were compared at baseline and after the end of the follow-up period of 4.0 ± 3.1 years. Kidney biopsy findings were evaluated using the Oxford classification. The evaluation of treatment included immunosuppressive therapy and renoprotection with angiotensin converting-enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB), or no treatment. The elevated serum IgA was found in 46 (52 %) patients and normal serum IgA level was found in 43 (48 %) patients. No differences were found between the two groups regarding the mean age of patients, proteinuria, and the number of patients with reduced GFR or HTN at baseline. In kidney biopsy, mesangial proliferation and segmental sclerosis were significantly more common in Group 1 compared with Group 2 (p < 0.05). Immunosuppressive therapy was used in 67 % children in Group 1 and 75 % children in Group 2. The Kaplan-Meier survival curves for renal function (with normal GFR) and persistent proteinuria did not differ significantly depending on the serum IgA level at baseline. We conclude that in IgA nephropathy the elevated serum IgA at baseline may be associated with mesangial proliferation and segmental sclerosis contribute to glomerulosclerosis, but has no effect on the presence of proteinuria or on the worsening of kidney function during several years of disease course.

Poor outcomes after liver transplantation in patients with incidental cholangiocarcinoma irrespective of tumor localization.

INTRODUCTION: After liver transplantation for cholangiocarcinoma (CCC), patients have a poor prognosis without use of specific therapeutic strategies. Accordingly, recipients with incidental CCC might have the highest risk of recurrent disease; however, sparse data on the long-term outcome of unselected patients with incidental CCC have been published. The aim of this study was to evaluate the post-transplantation outcomes of patients with incidental CCC with special focus on tumor localization. MATERIAL AND METHODS: There were 11 primary liver transplantations in patients with incidental CCC of 1310 liver transplantation procedures performed between December 1994 and August 2013. All patients with incidental CCC received a chemotherapy regiment including gemcitabine/5 fluorouracil, doxorubicin, and mitomycin. The patients were switched from calcineurin inhibitors to mammalian target of rapamycin inhibitor-based immunosuppression shortly after CCC diagnosis. RESULTS: Intra- and extrahepatic tumors were found in 6 and 5 patients, respectively. At median follow-up examination of 26.3 months there were 8 CCC recurrences and 7 patient deaths. Overall survival after liver transplantation for incidental CCC was 88.9% at 1 year, 44.4% at 2 years, and 14.8% at 3 years. The corresponding rates of recurrence-free survival were 45.7%, 45.7%, and 0.0%, respectively. Post-transplantation CCC recurrences were universal with 0% 3-year recurrence-free survival both in patients with intra- and extrahepatic tumors (P = .475). CONCLUSIONS: Incidental CCC in liver transplantation is associated with poor outcomes irrespective of tumor localization. Introduction of new adjuvant multimodal treatment concepts is necessary to improve the prognosis for this subgroup of patients.

Prediction of graft loss and death in patients with primary sclerosing cholangitis.

BACKGROUND: The prognosis of patients with primary sclerosing cholangitis (PSC) can be accurately determined using the Mayo Clinic Score (MRS), a mathematical model which predicts patient survival. The purpose of our study was to determine the risk of graft loss and/or death among patients who were listed or transplanted because of PSC. PATIENTS AND METHODS: We analyzed the data of 52 patients, who were placed on the transplant list due to PSC between January 2000 and November 2008 and either did or did not undergo liver transplantation (OLT). The primary end point (EP1) of the study was the patient death for any cause. The secondary end point (EP2) was recurrence of PSC or appearance of CCC or death related to the primary liver disease after OLT (PSC recurrence). The observation time was 60 months. According to the calculated MRS, patients were divided into 3 groups: group A (MRS < 0.56); group B (0.56 < or = MRS < 1.56), and group C (MRS > 1.56). The analysis was performed using the LIFETEST and PHREG Procedures of the SAS System. RESULTS: The risk of EP1 occurrence was 2.0 per 1 point of MRS (P < .0006). The risk of EP2 was 2.1 per 1 point of MRS (P < .001). Groups B and C compared with group A showed risks of death of: 0.79 (P = NS) and 6.59 (P < .08), respectively. The percentage of 5-year patient survival rate were 94%, 94%, and 45% according to groups A, B, and C, respectively. CONCLUSION: The risk of death in patients with MRS > 1.56 was 6.59-fold higher than those with MRS < 0.56. MRS > 1.56 significantly decreased 5 year survival among patients with primary sclerosing cholangitis.

A trans-activator on the Drosophila Y chromosome regulates gene expression in the male germ line.

The Y chromosome of Drosophila melanogaster accounts for approximately 13% of a normal male genome and is entirely heterochromatic. It carries six genes required exclusively for spermatogenesis. Here we report a novel activity of the Y chromosome that regulates gene expression in primary spermatocytes. By examining the expression of a reporter gene in X/Y and X/O males, we show that a specific region of the Y long arm carries a trans-activator that regulates transcription in spermatogenesis. In the absence of the Y trans-activator, the level of the reporter expression is greatly reduced in primary spermatocytes and the expression pattern is restricted to young primary spermatocytes. Further analysis shows that the Y trans-activator is dispersed in the h1-h10 region on the Y long arm and is functionally redundant, indicating involvement of the repetitive sequences on the Y chromosome. In addition, the Y trans-activator appears to act in a tissue-specific manner, functioning only in the male germ line. We propose that the Y trans-activator plays an important role in regulating gene expression during spermatogenesis.

Y-Linked male sterile mutations induced by P element in Drosophila melanogaster.

The Y chromosome in Drosophila melanogaster is composed of highly repetitive sequences and is essential only in the male germ line. We employed P-element insertional mutagenesis to induce male sterile mutations in the Y chromosome. By using a combination of two modifiers of position effect variegation, adding an extra Y chromosome and increasing temperature, we isolated 61 P(ry+) elements in the Y chromosome. Six of these Y-linked insertions (approximately 10%) induced male sterile mutations that are mapped to two genes on the long and one on the short arms of the Y chromosome. These mutations are revertible to the wild type in a cell-autonomous and germ-line-dependent manner, consistent with previously defined Y-linked gene functions. Phenotypes associated with these P-induced mutations are similar to those resulting from deletions of the Y chromosome regions corresponding to the male fertility genes. Three alleles of the kl-3 gene on the Y long arm result in loss of the axonemal outer dynein arms in the spermatid tail, while three ks-2 alleles on the Y short arm induce defects at early postmeiotic stages. The recovery of the ms(Y) mutations induced by single P-element insertions will facilitate our effort to understand the structural and functional properties of the Y chromosome.

[Yersinia enterocolitica infection in an 8-year old boy]. / Zakazenie spowodowane przez Yersinia Enterocolitica u 8-letniego chlopca.

A rare case is presented of Yersinia enterocolitica infection in an 8-year-old boy in whom symptoms and signs of appendicitis and cholecystitis dominated. Much attention is paid to the clinical course of yersiniosis and to diagnostic possibilities in order to identify the bacteria and to confirm the infection in serological tests.

Comparison of parenteral ciprofloxacin with clindamycin-gentamicin in the treatment of pelvic infection.

A prospective, randomized study of intravenous followed by oral ciprofloxacin compared with the combination of intravenous clindamycin and gentamicin was conducted in 122 women hospitalized with pelvic infections. Clinical diagnoses included endometritis (97 patients) and uncomplicated pelvic inflammatory disease (25 patients). Treatment successes for endometritis included 42 of 50 (84 percent) patients treated with ciprofloxacin compared with 35 of 47 (75 percent) treated with the clindamycin-gentamicin combination. Treatment successes for acute salpingitis included 10 of 10 (100 percent) treated with ciprofloxacin and 13 of 15 (87 percent) treated with clindamycin-gentamicin. Ciprofloxacin successfully eradicated Chlamydia trachomatis in 11 of 12 patients as did clindamycin-gentamicin in six of seven patients. In this study of pelvic infection, ciprofloxacin demonstrated efficacy comparable with the combination of clindamycin and gentamicin, and is effective against C. trachomatis.

Developing and implementing standards of practice for clinical pharmacy services.

The authors' department has attempted to bring more order to the provision and evaluation of clinical services through the development of "Clinical Standards of Practice." A pilot project was initially conducted on one satellite. Pharmacists were asked to prepare a list of minimum standards that could be agreed upon and that everyone believed were achievable even on the busiest days. After standards were developed and implemented, a procedure was established to evaluate compliance through review of pharmacy records and patient materials. Staff received feedback concerning the results of the review. As a result of the success with the pilot, Clinical Standards of Practice have been developed by staff and are in use throughout the department. Plans for the future include continuous revision of the standards to reflect changing departmental goals and directions and a change to a "pharmacist specific" rather than a current "nursing unit specific" monthly review format to facilitate performance review and staff development. Eleven standards are shown in the appendices.

Computerized attendance surveillance to reduce absenteeism.

Computerized data on absenteeism were used as a basis for employee counseling and disciplinary action, and the effect of this program on absenteeism was assessed. In the pharmacy department of an 1100-bed tertiary-care hospital, a microcomputer program provides weekly print-outs of number of occurrences of absence, reason for absence, and total hours absent for each employee. When an employee is within 10 absent hours or one occurrence of exceeding departmental absenteeism standards, these data are used in informal counseling. If absenteeism standards are exceeded, oral or written warning is given within one week after the violation. Absenteeism among pharmacists and technicians was compared for a 90-day period after implementation of this program and the same period during the year before implementation. The computerized attendance-surveillance program was associated with decreases in the mean number of absence occurrences for both pharmacists and technicians, fewer warnings issued to each group, and an increase in the mean number of hours worked.