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.

Management of maternal obesity prior to and during pregnancy.

The prevalence of obesity is high and rising worldwide. The greatest prevalence of obesity is found in the western world and in urban developing countries. There is an increased maternal mortality associated with maternal obesity. There are increased risks of most maternal complications in pregnancy including pre-eclampsia, gestational and pre-existing type 2 diabetes mellitus and thromboembolic disorders. There is an increased perinatal mortality associated with maternal obesity; there are increased risks of congenital malformation, fetal macrosomia and indeed risks for the fetus as a child and adult in the years to come. There are increased risks of complications of pregnancy including caesarean section, traumatic delivery and a reduced chance of breastfeeding. Maternal obesity in pregnancy predicts long-term risks for that mother. The management includes increased surveillance for these risks and lifestyle modulation during pregnancy. This includes dietary measures and encouraging modest increase in exercise. Ideally, the mother should achieve closer to an ideal body mass index prior to pregnancy using lifestyle intervention but possibly with pharmacological therapy or bariatric surgery. The ideal weight gain for an obese mother is less than the ideal weight gain for a lean mother.

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.

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.

Pigment synthesizing melanoma (so-called animal type melanoma): a clinicopathological study of 14 cases of a poorly known distinctive variant of melanoma.

AIMS: Pigment synthesizing melanoma (so-called animal type melanoma) (PSM) is a rare histopathological variant of melanoma so termed because of prominent melanin production and its similarity to a variant of melanoma seen in grey horses. The aim of this study was to report the clinicopathological characteristics of 14 cases of animal type melanoma. METHODS AND RESULTS: Six patients were female and eight were male with ages ranging from 5 to 52 years (mean 31 years, median 39 years). The head and neck represented the most common site. The clinical diagnosis was of melanoma in seven cases, blue naevus in three cases, benign naevus in three cases and a pigmented basal cell carcinoma in one case. The histological diagnosis of PSM was predicated on the basis of an asymmetrical, predominantly intradermal tumour formed of deeply pigmented, round or short, spindle-shaped dendritic melanocytes with some degree of hyperchromatism and a single nucleolus. Cytological atypia was always present but was not pronounced. A prominent population of macrophages was invariably present. Four tumours were compound and 10 tumours were predominantly intradermal. The mitotic count was usually low, ranging from 1 to 5 per 10 high-power fields (mean 2). Perineural and lymphovascular invasion was not seen. The Breslow thickness ranged from 1.1 to 7.5 mm (mean 3.3 mm). Follow-up was available in 13 patients. The median follow-up period was 5 years. Six patients had no recurrence, three had local recurrence in the form of satellite nodules adjacent to the scar, four had spread to the regional lymph nodes and one patient had distant metastases to the liver. There were no deaths. CONCLUSIONS: This study demonstrates that PSM is a distinctive, possible low-grade variant of melanoma usually lacking the histological features predictive of aggressive behaviour seen in ordinary melanoma. It should be managed in the same way as other melanomas with wide local excision.

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.

Common autosomal recessive diseases in Oman derived from a hospital-based registry.

OBJECTIVES: The local frequencies of genetic disorders in Oman apart from hemoglobinopathies are largely unknown. The aim of the present study was to evaluate birth prevalence of commonly diagnosed autosomal recessive diseases and to estimate needs and priorities of genetic services. METHODS: Analysis of the years 1993-2002 using a hospital-based registry of genetic diseases was attempted. More than 3,000 records were reviewed. Only patients with definite diagnosis were included in the analysis. Genetically determined diseases occurring less frequently than 1 in 50,000 births are not included. RESULTS: A number of rare autosomal recessive diseases are found to have a prevalence at least 1 in 50,000 livebirths. CONCLUSIONS: The data suggest that genetic diseases are important as major contributors to perinatal and childhood mortality and morbidity. The need for preventive genetic service is essential for the health of the community in Oman. Autosomal recessive diseases were frequently concentrated in specific geographical areas, which can be explained by founder effect and genetic drift. However, the hospital-based registry may present incomplete information. Further prospective studies are needed to provide more detailed data.

A retrospective hospital-based study on congenital hypothyroidism in the Sultanate of Oman.

A retrospective study was conducted on 45 children with congenital hypothyroidism (CH) to estimate the number of missed cases of CH among Omani children per year and compare the intellectual outcome of children diagnosed by neonatal screening (Group A) with those who were diagnosed clinically at a later age (Group B). Our results revealed 14 children in Group A, diagnosed at a mean age of 2.3 +/- 0.8 months and 31 children in Group B diagnosed at a mean age of 9.8 +/- 2.5 months. IQ assessment revealed that 67 per cent of the children in Group A had a normal IQ compared to only 15 per cent of those in Group B. The above results point to a great demand for a national screening programme in the Sultanate of Oman.

An unusual case of pneumonia.

A previously healthy 37-year-old man was admitted with a two-month history of increasing shortness of breath and high temperature. A chest X-ray demonstrated bibasal shadowing and small bilateral pleural effusions; arterial blood gases demonstrated low pO2. Despite intravenous antibiotics no significant improvement was observed. A high-resolution chest computed tomography showed diffuse ground-glass opacification with segmental and subsegmental airways opacification, indicating fine fibrosis. Subsequently, open lung biopsy showed diffuse alveolar damage and a histopathological diagnosis of acute interstitial pneumonia (Hamman-Rich syndrome) was made. Antibiotics were stopped and high intravenous doses of steroids were given with a dramatic improvement in the patient's breathing and radiographic findings. The pathophysiological mechanisms of acute interstitial pneumonia and current therapeutic options are 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.

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.

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.