Evaluation of oncology infusion pharmacy practices: A nationwide survey.

PURPOSE: Oncology care continues to evolve at a rapid pace including provision of infusion-based care. There is currently a lack of robust metrics around oncology infusion centers and pharmacy practice. The workgroup completed a nationwide survey to learn about oncology-based infusion pharmacy services offered. The objective was to highlight consistent, measureable oncology-based infusion pharmacy metrics that will provide a foundation to describe overall productivity including emphasis on high patient-safety standards. METHODS: A nationwide survey was developed via a workgroup within the Vizient Pharmacy Cancer Care Group beginning in April 2019 and conducted electronically via the Vizient Pharmacy Network from September to November 2020. The survey was designed to capture a number of key metrics related to oncology-based infusion pharmacy services. RESULTS: Forty-one sites responded to the survey. Responses highlighted hours of operation (median = 11.5), number of infusion chairs (median = 45). Staffing metrics included 7.1 pharmacist full-time equivalent (FTE) and 7.6 technician FTE per week. 80.5% of sites had cleanrooms and 95.1% reported both hazardous and nonhazardous compounding hoods. 68.3% of sites reported using intravenous (IV) technology, 50.0% measured turnaround time, and 31.4% prepared treatment medications in advance. CONCLUSION: There was variability among oncology infusion pharmacy practices in regard to survey responses among sites. The survey results highlight the need for standardization of established productivity metrics across oncology infusion pharmacies in order to improve efficiency and contain costs in the changing oncology landscape. The survey provides insight into oncology infusion pharmacy practices nationwide and provides information for pharmacy leaders to help guide their practices.

Wunderlichs syndrome in patient with sporadic bilateral angiomyolipomas - case report.

We present the case report of bilateral renal angiomyolipomas in a 70-year-old patient. Spontaneous bleeding into the retroperitoneum and hemodynamic instability required an acute surgical revision with a left-sided nephrectomy. The contralateral angiomyolipoma was selectively embolized in the second period and subsequently resected.

A novel CHST3 allele associated with spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.

Skeletal dysplasias (SDs) are highly heterogeneous disorders composed of 40 clinical sub-types that are part of 456 well-delineated syndromes in humans. Here, we enrolled consanguineous kindred from a remote area of Sindh province of Pakistan, with 14 affected individuals suffering with short stature, kyphoscoliosis, joint dislocations, clubfoot, heart valve anomalies and progressive bilateral mixed hearing loss. To identify pathogenic variants in this family, whole exome sequencing (WES) was performed in one affected and one normal individual, which revealed a novel transversion mutation (c.802G>T; p.Glu268*) in CHST3 associated with the phenotype. CHST3 encodes a chondroitin 6-O-sulfotransferase-1 (C6ST-1) enzyme that is essential for the sulfation of proteoglycans found in cartilages. Previously, mutations in CHST3 have largely been reported in sporadic cases of SD, primarily with severe spinal abnormalities, joint dislocations, joint contractures, and clubfoot. Clinical and radiological examination of the affected individuals in this family provides new insights into phenotypic spectrum of CHST3 alleles and disease progression with age.

Natural history of early first-trimester pregnancies implanted in Cesarean scars.

OBJECTIVE: To describe the ultrasound findings and natural history of pregnancies implanted within or on Cesarean section scars in the first trimester of pregnancy. METHODS: This was a prospective observational study of 10 women diagnosed with a pregnancy implanted in or on a Cesarean section scar in the first trimester, who declined medical intervention because of their desire to continue the pregnancy. The study population comprised women at < 12 weeks' gestation who were seen in our early pregnancy unit between January 2011 and September 2013. Nine women were followed up by serial ultrasound examinations and had detailed care plans for delivery at King's College Hospital (KCH). One woman was followed up and delivered at another teaching hospital. The first-trimester ultrasound findings were compared with the clinical outcome of the pregnancy. RESULTS: The nine patients who were followed up at KCH developed ultrasound findings of morbidly adherent placenta (MAP) in the second and third trimesters. All 10 patients were diagnosed with MAP at the time of delivery by Cesarean section. The gestational age at delivery ranged from 26 to 38 weeks. The uterus was conserved in five patients, and Cesarean hysterectomy was performed in the remaining five. All three women with complete implantation of the gestational sac within the scar and two of three cases with placental lakes in the first trimester had hysterectomies. The two cases with bulging of the gestational sac out of the uterine contour had a preterm emergency hysterectomy due to placenta percreta. Histology confirmed placenta accreta in the five hysterectomy specimens. There were no fetal or neonatal complications. CONCLUSIONS: Implantation of a pregnancy on or in a Cesarean section scar is a precursor of MAP; however, the degree of morbidity associated with this implantation is variable and difficult to predict based on first-trimester ultrasound findings only. The assessment of ongoing pregnancies implanted in Cesarean scars is most beneficial when performed between 7 and 9 weeks' gestation. Complete implantation within the myometrial defect, bulging of the trophoblast from the uterine contour and large placental lakes in the first trimester are ultrasound findings that may predict severe placenta accreta or percreta and consequently a poor outcome.

Age, gender, and racial differences in incidence and survival in primary CNS lymphoma.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma that accounts for ~4% of newly diagnosed central nervous system (CNS) tumours. The objective of this study was to analyse the epidemiology, incidence, and outcome of these rare tumours. METHODS: Primary brain and CNS lymphoma cases were identified from the Surveillance, Epidemiology, and End Results (SEER) research data sets for the years 1980-2008 for analysis of trends in incidence and survival. SEER(*)Stat v. 7.0.4 software was used to analyse the data. RESULTS: The overall incidence rate of PCNSL was 0.47 per 100,000 person-years. The incidence was significantly higher in males compared with females, blacks aged 0-49 years at diagnosis compared with whites, and whites aged 50 years and older at diagnosis compared with blacks. After a significant decline in incidence between 1995 and 1999, incidence rates rose slightly; those aged 75+ years at diagnosis had the most dramatic increase in incidence rates over time. Five-year survival rates were significantly higher in whites compared with blacks aged 0-49 years at diagnosis, but was primarily driven by white women aged 0-49 years. CONCLUSION: There is an increase in incidence of PCNSL in the elderly, and elderly blacks have lower incidence compared with white population. Survival remains poor and is negatively dominated by factors associated with HIV infection and advanced age.

Ecthyma gangrenosum: an important feature of pseudomonal sepsis in a previously well child.

Ecthyma gangrenosum is a rare, distinctive skin disorder associated with potentially fatal underlying pseudomonal sepsis. Although typically occurring in neutropenic or immunocompromised patients, it can occasionally affect healthy children. The appearances are characteristic with small indurated vesicular papules progressing rapidly to infarcted necrotic areas with surrounding erythema and a typical black eschar. In young children, these are often accompanied by fever and diarrhoea. The absence of suppuration and slough distinguishes it from the more recognized pyoderma gangrenosum. Lesions can occur at any site although are most commonly found over the buttocks, limbs, axillae and perineum. We describe the case of a 28-month-old, previously well child who presented with typical features of ecthyma gangrenosum secondary to Pseudomonas infection who responded to appropriate antibiotic treatment. Despite a thorough search, no underlying cause was found. Early recognition and prompt treatment with antipseudomonal antibiotics is vital to reduce morbidity and potential mortality.

Mid-term results of one-stage surgical correction of congenital vertical talus.

OBJECTIVE: Congenital vertical talus is a rare condition but a well-known cause of severe rigid flatfoot in children. The aim of this study was to evaluate the mid-term clinical and radiological results of one-stage surgical correction in children with congenital vertical talus. METHODS: Five feet in three children diagnosed with congenital vertical talus who had undergone surgical correction were followed up for a mean period of seven and half years. During this period they were clinically evaluated for subjective complaints and objective findings focused on the range of movement at the ankle joint, position of the hindfoot, and weight-bearing ability of the treated extremity. They were also evaluated on the basis of radiographs of foot and ankle made in standard projections. RESULTS: All the children had a good functional range of movement and normally shaped foot. The range of movement remains restricted and decreased during the follow-up period without causing any functional disability. All radiological measurements were within normal limits. There was no evidence of necrosis of talus. CONCLUSION: We recommend operative treatment for congenital vertical talus by the end of first year of age. The range of movement remains restricted and seems to decrease during follow-up, which had a little effect on the functional outcome of the ankle joint (Fig. 3, Tab. 1, Ref. 18). Full Text (Free, PDF) www.bmj.sk.

The changes of metabolic profile and weight during Ramadan fasting.

INTRODUCTION: Ramadan is the holiest month in the Islamic calendar and Muslims fast during this month. We designed this study to evaluate the effect of Ramadan fasting on plasma lipids and lipoproteins. METHODS: This cohort study was performed during Ramadan in December 2002 (Islamic year 1423). The subjects were 81 students of Tehran University of Medical Sciences. We evaluated weight, body mass index (BMI), glucose, triglyceride (TG), cholesterol, low density lipoprotein (LDL), high density lipoprotein (HDL), and very low density lipoprotein (VLDL), before and after Ramadan. RESULTS: Body weight and BMI both decreased during Ramadan fasting in both genders. Glucose and HDL decreased and LDL increased significantly during fasting in Ramadan, but there was no significant change in total cholesterol, TG and VLDL. We did not find any association between TG, cholesterol, LDL, VLDL, HDL and the following variables: sex, body weight changes, and two or three instances of meals before Ramadan. Triglyceride level also increased in students with normal BMI while it decreased in overweight subjects. CONCLUSION: This study indicated that Ramadan fasting led to a decrease in glucose and weight. Although there was a significant reduction in meal frequency, a significant increase in LDL and decrease in HDL was noted during Ramadan. It seems that the effect of Ramadan fasting on serum lipid levels may be closely related to the nutritional diet or biochemical response to starvation.

Penetration of anticancer drugs through solid tissue: a factor that limits the effectiveness of chemotherapy for solid tumors.

Penetration of anticancer agents to cells distant from the vascular system is required for efficacy of cancer chemotherapy against solid tumors. Many solid tumors have a poorly formed blood vascular system with variable rates of blood flow and much larger intercapillary distances than those found in normal tissues. The requirement for drugs to penetrate several layers of tissue might pose a barrier to the effective treatment of solid tumors. Multicellular layers (approximately 200 microm thick) were grown in vitro on Teflon membranes from EMT6 murine and MCF7 human tumors and have been used to quantitate the penetration of four widely used anticancer drugs through solid tissue. The penetration of doxorubicin and mitoxantrone was limited and very slow (<10% of the rate of penetration through the Teflon membrane alone). The penetration of methotrexate and 5-FU was more rapid (approximately 30-50% of the rate of penetration through the Teflon membrane alone), but remains a substantial barrier to the effectiveness of these drugs. Strategies to improve the penetration of anticancer drugs through poorly vascularized tumor tissue have considerable potential to improve the outcome of chemotherapy for solid tumors.

Fatal donor-derived Epstein-Barr virus-associated post-transplant lymphoproliferative disorder following reduced intensity volunteer-unrelated bone marrow transplant for myelodysplastic syndrome.

Post-transplant lymphoproliferative disorders (PTLD) are a well recognised complication of conventional haemopoietic stem cell transplantation (HSCT). Reduced intensity HSCT involves intensive immunosuppression to permit engraftment. Thirty reduced intensity transplants with the FBC (fludarabine 150 mg/m2, busulphan 8 mg/m2, CAMPATH-1H 100 mg) protocol have been performed at our centre, with one confirmed EBV-positive PTLD. The female recipient developed a perforated viscus day +191 following HSCT from a volunteer unrelated male donor. A large caecal mass and a retroperitoneal abscess were excised, revealing an EBV-positive diffuse large B cell lymphoma confirmed by FISH to be of donor origin. More experience is required before the risk of PTLD in this setting can be assessed.

Adenomyosis in Pakistani women: four year experience at the Aga Khan University Medical Centre, Karachi.

As part of a quality assurance programme at the Aga Khan University Medical Centre, Karachi, Pakistan, all hysterectomy specimens were reviewed from January 1986 to December 1989. Adenomyosis was found in 237 of the 419 (56.5%) specimens studied. Of these 237 patients, 232 (97.9%) were parous and 196 (82.8%) were in the fourth and fifth decades of life. This high prevalence in parous women aged 40-59 years was significant. Fibroids, cervicitis, and endometrial hyperplasia were the most common associated diagnoses. Of all the associations studied, only endometrial hyperplasia was significantly more prevalent in the group with adenomyosis. Adenomyosis was stated as an indication for surgery in 69 patients and was confirmed by histopathology in 49 (71%). Preoperative suspicion of adenomyosis was present in 49 (20.6%) patients of all those ultimately found to have the disease. There is a high prevalence of adenomyosis in the population studied, which indicates that the condition may have been underdiagnosed in the past, especially as it is difficult to diagnose without surgery and hysterectomy is currently the only treatment.

Prevalence and pathology of helminth parasites in domestic ducks of Bangladesh.

Parasitism in waterfowl is a very common phenomenon. Two detailed check lists of helminth parasites of waterfowl are available (Lapage, 1961; McDonald, 1969) and many of these parasites are reported to occur in domestic ducks. However, the pathological significance of most of them is unclear. In a preliminary study, Qadir (1979) recorded 13 species of helminths from domestic ducks of Bangladesh. The present paper reports on the prevalence and pathological effects of helminth infections in domestic ducks (Anas platyrhynchos domesticus) of Bangladesh under natural conditions and on their incidence in two age and sex groups of ducks.

The effect of caffeine on normal and denervated gastrocnemius muscles of Uromastix hardwickii.

The effect of time and 5 mM caffeine was studied on the maximum twitch tension of the normal and 11-13 days denervated gastrocnemius muscles of Uromastix hardwickii. The maximum twitch tension was found to decrease with time and it remained about 68% of initial after 60 minutes at 20 degrees C. Five mM caffeine only produced potentiation in normal and denervated muscles while there was no contracture in either muscle. Although caffeine increased the overall contraction and relaxation times in control muscles, it was more effective on the contraction time of the denervated muscles in which the contraction time increased with time. Denervated muscles were seen to be less sensitive to caffeine as the contraction times increased with time (at about 30-40 minutes) in these muscles while in normal control muscles the contraction time increased immediately. The action of caffeine in relation to these effects is discussed.

Spontaneous improvement of severe neonatal hyperparathyroidism after failed total parathyroidectomy.

We report an interesting case of severe neonatal hyperparathyroidism (SNHP) treated by sub-total parathyroidectomy, as her fourth parathyroid gland could not be identified. The clinical course changed over time to a more benign course resembling familial hypocalciuric hypercalcemia. This indicates that the natural course of SNHP is probably gradual spontaneous improvement, if patient survives the first few months of life.

Amoebic appendicitis--a rare entity.

Appendectomy is the most common surgical procedure performed in routine general surgical practice. However, not all the appendices removed, are submitted for histopathological examination in this part of the world. We reviewed 1400 appendices received by our department from within our hospital and from outside the hospital. Of these 13 cases were reported as amoebic appendicitis. These patients did not have any different clinical presentation from the patients who were reported as acute appendicitis without amoebae. Microscopically these appendices had minimal neutrophil polymorph infiltration accompanied by tissue necrosis and amoebic trophozoites within the appendiceal wall. After histological diagnosis, different tests (IHA and stool examination) were done to exclude a possibility of secondary involvement of the appendix, on 8 patients from our hospital which were negative, thus confirming that these patients had primary appendiceal involvement. Hence we recommend that all the appendices removed should be subjected for histological examination, since ths may help in subsequent management of these patients.

Is carcinoma breast a different disease in Pakistani population?

Carcinoma of the breast tends to be different in coloured and white races. Is this pattern also expressed in Pakistani population? To answer this query we carried out a retrospectively study of breast cancer on 193 cases who were divided into 2 groups i.e. less than and more than 50 years age groups. In the former group, 93% tumours were of grades II or III and approximately 51% were estrogen receptors negative. In more than 50 years age group, 75% tumors were in grade II and III, with almost 37% being estrogen negative tumors. Majority (75%) of the patients had over 6 cms lump with equal number having positive lymph node status. All these factors point to the fact that besides presenting late, our population has additional unfavourable prognostic factors.

Immunohistochemical estrogen receptor determination in human breast carcinoma: correlation with histologic differentiation and age of the patients.

An immunohistochemical assay for the measurement of estrogen receptor (ER) has been evaluated on 290 consecutive human breast biopsy and mastectomy specimens in the year 1992 at The Aga Khan University Hospital laboratories. Immunohistochemical localization of estrogen receptor on frozen/paraffin section was scored in a semi-quantitative fashion incorporating both the intensity and the distribution of specific staining. Histologic grading of the tumour was performed according to Bloom's method. In this study, 21% of the tumours were estrogen receptor negative, 15% were weak positive, 25% intermediate positive and 39% strong positive. Fifty percent of the well differentiated tumours showed strong ER positivity against 27% of the poorly differentiated tumours. Seventy eight percent of all negative estrogen receptors were in patients younger than 50 years of age (pre-menopausal group), while 52% of strong estrogen receptor positivity was observed in patients older than 50 years (post- menopausal). This study demonstrates the value of immunohistochemical method to determine the ER status in patients with advanced breast cancer.

Yet another cause of chronic viral hepatitis?

The clinical features, course and histology of liver in 20 patients; mostly middle aged to elderly females, closely resembling chronic Non A Non B hepatitis is presented. They presented quite late in their disease and therefore, complications such as variceal bleeds, ascites and encephalopathy were frequent. Our patients were negative for hepatitis B and C virus serology. Metabolic and immune causes of chronic liver disease were also ruled out. To the best of our knowledge, this is the first study of its kind elaborating the clinical features, course and histology of liver in chronic Non B Non C hepatitis and raises a number of questions as to the nature of the infecting virus and the epidemiology of disease.

Immuno-histochemical analysis of estrogen receptors on the malignant gallbladder tissue.

Sex steroids play an important part in the functioning of normal gallbladder, formation of gallstones and possibly in the pathogenesis of gallbladder cancer. Steroids receptors have been previously demonstrated on normal and malignant gall bladder tissues. To study this phenomenon further, we correlated clinicopathological features and survival with estrogen receptor (ER) status of the tumour in 30 patients with histologically-proven adenocarcinoma of the gallbladder. Estrogen receptor assay was performed immuno-histochemically utilizing Universal Immunoperoxidase Staining Kit. Tumor tissue was obtained either surgically or with fine needle aspiration of the gallbladder mass. There were 27 females and 3 males. Eighteen patients had estrogen receptors expressed on the malignant tissue, 12 were negative. Comparison of clinicopathological characteristics and survival between the two groups demonstrated no significant difference in gender, mean age, marital status and parity. Similarly, presence of gallstones, histologic grade or survival did not correlate with the estrogen receptor status. There is, however, a trend in favour of poorly differentiated tumors being more often receptor negative. Further studies are necessary to elucidate the biologic significance of these receptors.

Changing patterns and re-distribution of antigen in poorly differentiated carcinomas: its implications in tumour diagnosis.

The variability of expression of tumour-associated antigens via either antigenic heterogeneity or antigenic modulation presents a basic problem in immunohistochemical diagnosis of poorly/undifferentiated tumours. This work was designed to study antigenic expression on human resected epithelial tumours by a panel of most widely used antibodies (EMA, CEA, AUAI & Cytokeratin) in relation to tumour differentiation and polarization. It was observed that poorly differentiated carcinoma with loss of polarity show homogeneous membrane staining (with antibodies against EMA, CEA & AUAI) in contrast to either apical (luminal) or basolateral membrane staining in well differentiated counterparts. Biochemical studies have shown that apical and basolateral epithelial cell membrane domains have a characteristic set of glycoproteins. Tight junctions are essential for maintaining this functional polarization. It was concluded that structural and functional abnormalities of tight junctions in poorly differentiated carcinomas results in loss of polarity with progressive invasion of the cell surface by antigenic glycoprotein and resultant homogeneous individual cell antigenic expression in poorly differentiated carcinomas. This study demonstrates that antigenic expression on tumour cells is not static, but dynamic and heterogeneity of antigenic expression may well be due to biological factors such as spatial configuration of the lesion.