Jeff Kushner, PhD
- Associate Professor of ISAT
- College of Integrated Science and Technology
- James Madison University
- Harrisonburg, Virginia
Pain is whatever the experiencing person says it is existing whenever the experiencing person says it does (McCafery virus spreading in us buy discount bactrim, 1968) antimicrobial drug resistance discount 480mg bactrim overnight delivery. Physical Social Psychological Total Pain Spiritual Pain is one of the most frequent and serious symptoms experienced by patients in need of palliative care antibiotic vitamins order bactrim 960mg with amex. The goal is to prevent or treat as early as possible the symptoms of a disease can you take antibiotics for sinus infection while pregnant purchase 480mg bactrim otc, side efects caused by treatment of a disease antibiotics for acne trimethoprim purchase bactrim 960mg on-line, and psychological do antibiotics for acne work bactrim 480 mg for sale, social and spiritual problems related to a disease or its treatment. Integrating Palliative Care in Oncology: the Oncologist as a Primary Palliative Care Provider. In Silbermann, M; Palliative Care: Perspectives, Practices and Impact on Quality of Life. Care of Patients at the End of Life: Surrogate Decision Making for Incapacitated Patients. Planning and implementing palliative care services: A guide for programme managers. It covers the physical, psychosocial and economic issues of cancer, beyond the diagnosis and treatment phases. It includes issues related to the ability to get health care and follow-up treatment, rehabilitation, surveillance for late efects of treatment, screening for recurrence & secondary cancers and quality of life. Family members, friends and caregivers are also considered part of the survivorship experience. Surveillance for recurrence/spread of cancer and screening for subsequent primary cancers at least annually. Interventions for consequences of cancer treatment such as pain and peripheral neuropathy management, lymphedema management, for those who received anthracyclines, assess for anthracycline-induced cardiotoxicity (evaluate for heart failure signs and symptoms, presence of risk factors), need for immunizations. Care co-ordination between specialists and primary care givers with specifc roles delineated to ensure the health needs of the cancer survivor are met. Survivorship care planning: this plan will include a summary of treatment received, follow-up care, surveillance and screening recommended for the survivor, post-treatment needs of the survivor, healthy lifestyle behavior recommendations and information on treatment related side efects and anticipated health risks. Cancer Survivorship Issues: Life after Treatment and Implications for an Aging Population. American Society of Clinical Oncology expert statement on cancer survivorship care planning. Breast cancer tends to occur at a relatively young age (35-50 years) in Kenya, in comparison to Western countries (50-55 years). About 90% of cases occur sporadically, while only 5-10% can be attributed to genetic predisposition. Mammography and ultrasound fndings, category and recommendations are as tabulated below: Table 2. Other disciplines, such as plastic and reconstructive surgeons, speech therapists, occupational/physiotherapists may be included as and when need rises. Various classifcation systems use a combination of these factors to categorize patients into risk factor and prognostic groups. However, due to the heterogeneous nature of breast cancer, individual patients in the same risk category may have diferent clinical outcomes. Thus, the advent of molecular classifcation in recent years has seen a refnement of the traditional classifcation system to better assess the prognosis and predict response to therapy of Breast Cancer at an individual level. Immunohistochemistry has been used as a surrogate marker for the intrinsic Breast Cancer subtypes as defned by gene expression profling. The drain site should be anterior to the mid axillary line to enable coverage of site by radiotherapy. Chemotherapy Protocols Adjuvant chemotherapy should be started as soon as clinically possible, usually within 6 weeks of completion of surgery. Choice of the regime should be left to the oncologist dependent on the patient characteristics, tumour characteristics and system/social-economic characteristics, among others. Repeat every 21 days x 6 cycles o Doxorubicin/Cyclophosphamide x 4 cycles every 21 days followed by Taxane x 4 cycles every 21 days. Patients on bisphosphonates should be on calcium supplements with monthly monitoring of kidney function. The need for regular follow up for timely detection and salvage of recurrences, treatment of contralateral/metastatic disease and management of late sequelae of treatment including quality of life and survivorship plan should be emphasised. Take a detailed history metastatic symptoms, physical / psychosocial sequelae of treatment 2. The follow-up frequency should be: 3 monthly for 2 years; 6-monthly for 3 years; then annually. Lymphedema management: often caused by the interruption of regional lymphatic drainage. Circumferential measurements of both extremities should be taken at the metacarpal-phalangeal joints, the wrists, 10 cm distal and 15 cm proximal to the lateral epicondyles at baseline and after treatment. Symptoms will include swelling on the same side of treatment, sensation of heaviness of the limb, fatigue, fullness/tightness of skin or pain. Options of care include: external compression garments (such as lymphedema stockings), massage/ manual lymphatic drainage, elevation, exercise, psychosocial support and prompt treatment of infections. Breast reconstruction: Every patient undergoing mastectomy should be informed of the option of breast reconstruction for patients with early breast cancer or locally advanced breast cancer as appropriate. Breast prostheses may be considered for breast cancers survivors who have undergone mastectomy. Management of hormone-related symptoms: menopause management in females by appropriate specialist. Sexuality support and management should be discussed and if needed patient sent to experts for management. Classifcation and prognosis of invasive breast cancer: from morphology to molecular taxonomy. The annual global age-standardized incidence of primary malignant brain tumors is ~3. In adults, two thirds of primary brain tumors arise from supratentorial region with gliomas, metastases, meningiomas, pituitary adenomas and acoustic neuromas accounting for 95% of all brain tumors. The referring health care provider should inform the health care provider at the receiving facility about the referral and write a comprehensive medical report to accompany the patient including all scans done. Biopsy: Tissue obtained at emergency or elective surgery should be submitted to histopathology for examination. Diagnostic biopsy is required before initiation of any chemotherapy or radio therapy, except for high-risk cases where biopsy cannot be done and emergency radiotherapy is required. Immunohistochemistry is recommended for confrmation of diagnosis, being mandatory for high-grade or equivocal tumors where the histogenesis is unclear. For long term treatment, carbamazepine or lamotrigine may be used for focal onset seizures and sodium valproate or lamotrigine for primary generalized seizures. Defnitive Management Maximal safe debulking surgery is the initial standard of care to relieve mass efect, obtain diagnostic tissue, reduce tumor burden and to improve or maintain neurological status. Confirmed histological diagnosis and oncology review should be within ten days of surgery due to the rapid doubling time of the tumor. Adjuvant treatment such as radiotherapy with concurrent chemotherapy may be required. Recurrent Disease/ Progression Surgery is indicated in selected patients to relieve symptoms, improve performance status and quality of life. Repeat radiotherapy may be considered, depending on size of lesion, previous dose and the interval since the last radiotherapy treatment. Active agents include Carmustine, Vincristine, Temozolomide, Irinotecan and Bevacizumab. Thereafter scans are usually done at 6 months and then annually, or if clinically indicated. Management Surgery is the primary treatment for most pituitary tumors (except prolactinomas which may be managed medically). Radiotherapy is indicated for sub totally resected tumors, recurrent tumors, patients with persistently elevated circulating hormone levels, and medically inoperable patients. It is very efective for control of growth of pituitary tumors (>95%), but is less efective for decreasing circulating hormone levels of endocrinologically active tumors whose control may take years to achieve after radiotherapy. Possible etiologies include previous exposure to ionizing radiation, trauma, viral infections and exposure to sex hormones (approx. Imaging may give an indication as to the grade of the meningioma with the lesions with predominant surrounding edema being higher grade. Radiotherapy Post-operative radiotherapy signifcantly improves survival rates and is standard of care. The dose of radiotherapy given is dependent on the grade of meningioma, extent of resection +/residual disease and site (proximity to dose limiting structures). External beam radiotherapy is used for treatment with few indications for the use of stereotactic radiosurgery in small tumors located next to eloquent areas. Tumors that commonly present with brain metastasis include, but are not limited to lung cancer, malignant melanoma, breast and prostate cancer. Defnitive Management Surgery can be of ered to resect solitary brain metastasis or multiple brain metastases with a controlled primary tumor. Whole brain radiotherapy can be ofered but this results in signifcant neurocognitive decline that needs to be discussed with the patient before the treatment. Observation for asymptomatic patients especially grade I lesions without immediate intervention. Mechanical back painvaries with movement and spinal position is due to Spinal column instability 3. Spinal cord compression accounts for 5-10% of presentation in oncology patients and can be debilitating especially with prolonged symptoms. Management of Spinal Cord Metastasis Palliative therapy is mostly instituted and depends on performance status, duration of cord compression, site(s) of involvement, structural integrity of the spine and available resources. Despite their diferences in management, both primary and secondary brain tumor patients experience similar symptom morbidity that impacts their quality of life. By setting realistic goals, they can have a better sense of control and reduce their dependency on others. Cancer rehabilitation goals are not universal but should be set according to the prognosis of each patient. After brain tumor resection, for instance, patients may have cognitive defcits for which they can be taught to compensate with therapy. Brain Cancer Symptoms Intracranial Other Pressure Insomnia Altered Cognative (Depending causing Seizures and Mental Impairment on Lesion headaches Fatigue Status Location) Corticosteriods Psychosocial Psychosocial (Dexamethasome) Anticonvulsants No Interventions Cognitive Evidence Interventions -and/or---or---and/or Rehabilitation Yet -and/or Analgesics Benzodiazepines Pharmacological Relevannt Interventions. Epidemiology of primary brain tumours: Current concepts and review of the literature. Randomised comparison of stereotactic radiosurgery followed by conventional radiotherapy with carmustine with conventional radiotherapy for patients with glioblastoma mul tiforme: Report of Radiation Therapy Oncology Group 93-05 protocol. Overview: Brain Tumour Diagnosis and Management/Royal College Of Physicians Guidelines. For patients who are potentially curative consider chemoradiation prior to stenting. Follow Up Follow-up visits should be concentrated on symptoms management, nutrition and psychosocial support. The incidence of oesophageal cancer in Eastern Africa: identifcation of a new geographic hot spot It is the 3rd most common gastrointestinal malignancy after esophageal and colorectal cancers.


Both modalities are secondary mutations include deletion of 17p13 (the p53 important for accurately establishing patient prognosis infection jaw buy bactrim on line. Recently virus 3030 purchase genuine bactrim line, a schema has been developed that stratifies Monoclonal multiple myeloma into high- bacteria klebsiella discount 960mg bactrim mastercard, intermediate- antibiotic 3 day dose buy bactrim online from canada, and standardGammopathy Criteria risk disease (Table 27 infection 4 the day after best 480mg bactrim. In addition antibiotics for dogs with salivary gland infection purchase bactrim toronto, the presence of plasma cell leukemia Monoclonal gammo(A) M-protein in serum <3. If the serum and urine M-protein are unmeasurable and serum-free light assay is also unmeasurable, 50% reduction in plasma cells is required in place of M-protein, provided baseline bone marrow plasma cell percentage was 30%. In addition to the previously listed criteria, if present at baseline, a 50% reduction in the size of soft tissue plasmacytomas is also required. Definite development of new bone lesions or soft tissue plasmacytomas or definite increase in the size of existing bone lesions or soft tissue plasmacytomas. It is not used in calculation of time to progression or progression-free survival but is listed here as something that can be reported optionally for use in clinical practice. A definite increase is defined as a 50% (and at least 1 cm) increase as measured serially by the sum of the products of the cross-diameters of the measurable lesion. Asymptomatic (Smoldering Myeloma) Response to treatment in multiple myeloma is defined by the International Uniform Response Criteria defined A. These criteria are summarized in nation was noted to have an elevated serum protein level 340 Tumor Board Review of 9. This abnormal protein level then prompted an 2-microglobulin and quantitative Igs, serum protein evaluation of multiple myeloma. The following laboratory electrophoresis, serum immunofixation/electrophoresis, results were obtained: serum-free light chain assay, urine protein electrophoresis, and urine immunofixation/electrophoresis. A skeletal surWhite blood count 6700/L vey may be included annually in the assessment and a bone Hemoglobin 14. Platelets 218, 000/L However, once the patient meets the criteria for symptomCalcium 9. The serum protein for Autologous Stem Cell Transplant electrophoresis and immunofixation demonstrated an R. Laboratory evaluation was remarkable assay was obtained and showed a kappa-free light value for mild normochromic, normocytic anemia (hemoglobin of 0. A comprehensive metabolic panel showed Serum quantitative Igs were performed that demonstrated only mildly decreased albumin at 3. IgA level was an elevated IgA level of 2990, a normal IgG level of 51, elevated at 3. Serum-free light chain degenerative changes but otherwise no evidence of lytic analysis was positive for elevated monoclonal lambda lesions. We also observe that his calcium, must be considered, as they impact therapy selection. Adjunctive therapy targeting the complications of when compared to those observed with traditional chemyeloma and its treatment should span all phases of dismotherapy regimens, such as vincristine/doxorubicin/ ease management. While 3-drug regimens tend to induce anthracyclines, vincristine, and high-dose steroids have superior responses to those seen with 2-drug combinabeen a backbone of myeloma regimens for decades. These drugs have not only tion into high-, intermediate-, and standard-risk cohorts led significant improvement in response to therapy, but (Table 27. If lenalidomide-containing Thalidomide or Lenalidomide regimen is used for >4 cycles, particularly in patients older than 65 years, stem cell collection should occur with use Risk Factor Recommended Action of stem cell mobilization agents (cyclophosphamide with Individual risk factor If no risk factor or any 1 risk filgrastim or plerixafor) (12). Hyperviscosity is another major complication of multiple myeloma occurring in selective patients and often presenting as a medical particularly those with poor responses to primary therapy emergency. In patients with myeloma-related renal resulting low transplant-related morbidity and mortality. On laboratory evaluation, IgG kappa was elevated at Transplant-Ineligible Patients 3. Lenalidomide is the only medication to tetrasomy 9, indicating a hyperdyploid clone. Bortezomib maintenance has also Evidence-Based Case Discussion been shown to deepen the response rates, however, to this Ms. Carfilzomib showed promIgG value of 3600 mg/dL, an IgA value of 96 mg/dL, and ising clinical activity in relapsed and relapsed/refractory decreased IgM value of 24 mg/dL. A skeletal survey showed multiple myeloma with a substantially lower incidence of new osteolytic lesions in the bilateral humeri. A bone marperipheral neuropathy compared to bortezomib, and when row biopsy revealed an increase to 17% plasma cells conemployed in a combination with lenalidomide and dexamsistent with relapsed disease. Various carfilzomib-based combinations are currently hyperdiploid clone with trisomy 3, 7, 9, 11, and 15. There has been an increase of promising new therarenal failure, hypercalcemia, or lytic bone lesions. A 60-year-old man with multiple myeloma harborother approaches including alkylating agents, deacetylase ing t(11;14) achieved a complete remission after inducinhibitors, signaling transduction pathway inhibitors, and tion therapy with cyclophosphamide, bortezomib, and tyrosine kinase inhibitors are ongoing. He is now presenting with a significantly increasing M-protein and evidence of increased plasma cells in 1. A 64-year-old man presents with hypercalcemia, acute his bone marrow, consistent with relapse. Which of the following is the next appropriate step Which of the following is associated with high-risk for his relapsed multiple myeloma A 50-year-old man is found to have a serum monoclo(D) Continue maintenance therapy until he has evinal (M) protein of 1. He does not demonstrate any anemia, mia, renal failure, or skeletal lytic lesions) 346 Tumor Board Review 4. He is presenting to response, plans for autologous stem cell transplant the clinic prior to his next cycle of chemotherapy. Stem cell collection is performed after 4 cycles of to starting his last cycle was 56. A 67-year-old obese but generally healthy man sustherapy is equally high for patients on lenalidotained a right hip fracture in a fall. Imaging demonmide and thalidomide strated several lytic lesions in the affected limb and (C) Bortezomib maintenance should only be used concern for multiple myeloma was raised. Complete if bortezomib was part of the primary therapy blood count with platelets and differential and comregimen prehensive metabolic panel were remarkable only for (D) Maintenance therapy should not be pursued for an albumin of 3. Free serum lambda the duration of maintenance therapy light chain was found to be elevated to 174. Bone marrow biopsy International Staging System IgA kappa multiple showed 60% atypical plasma cells and adjunct cytomyeloma, based on antecedent monoclonal gammogenetic studies identified presence of t(4;14). Which of pathy of undetermined significance diagnosed when the following primary therapeutic regimens are you she was 58 years. Cytogenetic analysis performed on (C) Melphalan/prednisone/bortezomib bone marrow biopsy specimen identified only trisomy (D) Bortezomib/cyclophosphamide/dexamethasone 8. While she is activities-of-daily-living and instru(E) Vincristine/liposomal doxorubicin/dexamethasone mental activities of daily living independent, she has a past medical history of well-controlled non-insulin6. The same patient in Question 5 presents to the clinic dependent diabetes, hypertension, dyslipidemia, and to discuss treatment options. International uniform response criteplete recovery from hip replacement ria for multiple myeloma. The molecular characreactivation terization and clinical management of multiple myeloma in the post-genome era. Second malignancies plus dexamethasone is superior to vincristine plus doxorubicin in total therapy 2 and 3 for newly diagnosed multiple myeloma: plus dexamethasone as induction treatment prior to autologous influence of thalidomide and lenalidomide during maintenance. Cyclophosphamide, phalan-prednisone-thalidomide and melphalan-prednisone for bortezomib and dexamethasone induction for newly diagnosed the first line treatment of multiple myeloma. Incorporating borteContinuous lenalidomide treatment for newly diagnosed multiple zomib into upfront treatment for multiple myeloma: early results myeloma. Prevention of thalidomideand lenalidotage, in relapsed/refractory mm: a phase 3, multicenter, randommide-associated thrombosis in myeloma. Multiple environmencell that alter regulation of cellular proliferation, differtal factors, including nuclear, electromagnetic, and cosentiation, and apoptosis. Neutropenia may be associated with life-threatening require alternative and/or more intensive treatment and bacterial or viral infection. Clinical findings include nonbulky lymphadeGene expression profiling studies also enable classinopathy, hepatosplenomegaly, petechiae, or pallor. This is seen in patients with specific translocations resulting in overactivation of the interleukin-3 gene on chromosome 5 and eosinophil proliferation. Typically, the B-cell precursor lymphoother translocations has prognostic significance and may blasts are large with sparse cytoplasm and an enlarged determine whether patients are referred for transplantation nucleus, with fine chromatin and poorly defined nuclein first or second remission. This patient has none for pediatric and adult populations are presented in Tables of the usual adverse risk features in adults with B-cell pre28. Improved treatment has negated hematopoiesis, which enables administration of further the prognostic significance of previously identified highintensive chemotherapy. Adequate dose intensity is an important >1% factor associated with decreased relapse in maintenance Standard risk All others phase (10). The reinduction regimens use the same drugs that were initially used to achieve induction. Reduction in the duration of maintenance therapy Routine antiviral and pneumocystis jiroveci prophylaxis results in higher relapse rates. Consensus transfusions for clinically significant anemia and platelet guidelines from pediatric (but not adult literature) transfusions for platelet counts <10, 000/L. Other monoclonal antibodies that are under investirevealed normocellular marrow with no identifiable blasts. This was followed by 3 years of maintenance with tional chemotherapy or other monoclonal antibodies. He tolerated and was diagnosed with Pseudomonas pneumonia on day therapy well, except for an episode of transaminitis during 10 of induction chemotherapy. He is currently 4 years from initial diagnosis broad-spectrum antibiotics for a period of 5 days. He continues to follow-up for clinical course improved as his blood counts recovered. Bone marrow aspiration on day 24 revealed 40% cellularity with no discernible blasts on morphological examination. Relapse is With Adverse Features thought to be due to residual leukemia cells that are below M. Initial studies showed survival benefit (>30, 000/L), which are poor prognostic factors. This patient allogeneic transplantation approximately 2 months after should be treated using standard induction therapy with initial diagnosis. He had mild, acute, and chronic graft steroids, vincristine, anthracycline, and cyclophosphversus host disease posttransplantation. High-dose methotrexate should be used with biopsies on days 90 and 365 posttransplant showed comcaution and may require dose reduction. There is evidence that incorporation of rituximab of vincristine and dexamethasone (21). Cytogenetics from the bone marrow specimen astinal mass was visible on chest x-ray. Nelarabine has efficacy in Newer data are emerging concerning the use of dasatinib, the relapsed setting and has been evaluated in the frontline which may be a more potent drug as it inhibits multiple setting (26). Imatinib was started on day 1 of induction and dation and maintenance therapy for a total of 3 years. The patient achieved hematological and cytogenetic remission after cycle 1 of induction chemotherapy. A 35-year-old man presents with 1 month of increastook 400 mg of imatinib daily for 24 months after transing fatigue. It is questionable whether there is any benefit of cyte count of 23, 000/L, hemoglobin of 5. Which of the following is considered to be a sor B-cell acute lymphoblastic leukemia and the good prognostic factor for above patient

On the other hand virus vaccines buy generic bactrim on line, muscle tissue from patients with Becker muscular dystrophy contains reduced amounts of dystrophin or antibiotic resistance environment generic 960mg bactrim, occasionally virus 1 cheap bactrim 480mg with mastercard, a protein of abnormal size finished antibiotics for uti still have symptoms discount bactrim 480mg mastercard. Is corticosteroid therapy effective for the treatment of Duchenne muscular dystrophy Several studies have documented an improvement in strength with an optimal dose of prednisone of 0 staph infection buy 480mg bactrim with mastercard. Appropriate timing and duration of treatment have not been established antibiotics uti cheap 960 mg bactrim overnight delivery, and side effects (weight gain and increased susceptibility to infection) may outweigh the benefits in many cases. What is the most likely diagnosis in a child with progressive walking difficulties evolving over several days It is frequently preceded by a viral respiratory or gastrointestinal illness and rarely by surgery or immunizations. As a result of the loss of the healthy myelin covering, the conduction of nerve impulses (action potentials) may be blocked or dispersed. Some individuals have mild brief weakness, whereas fulminant paralysis occurs in others. The Miller Fisher variant is characterized by gait ataxia, areflexia, and ophthalmoparesis. Most common infections or inflammatory processes generate an elevation of white blood cell count and protein. Early clinical monitoring is focused on the development of bulbar or respiratory insufficiency. Bulbar weakness manifests as unilateral or bilateral facial weakness, diplopia, hoarseness, drooling, depressed gag reflex, or dysphagia. Frank respiratory insufficiency may be preceded by air hunger, dyspnea, or a soft muffled voice (hypophonia). The autonomic nervous system is occasionally involved, and this is signified by the presence of labile blood pressure and body temperature. Hydration is maintained intravenously, and nutritional support is provided by nasogastric feedings. Careful pulmonary toilet is conducted to minimize atelectasis, aspiration, and pneumonia. In rare cases, the neuropathy may recur as a chronic inflammatory demyelinating polyneuropathy. Studies of affected children demonstrate a variable predominance of boys during early childhood and females during adolescence. Ataxia, muscle weakness, and transient visual or sensory T G uillain arre F eature Tick P aralysis yn drom e pin alC ord L esion P oliom yelitis A taxia Pre se nt bse nt bse nt bse nt Rate ofprogression ours to d ays ays to we ks G rad ualor abrupt ays to we ks Musclestretch bse nt bse nt Variabl bse nt reflexes B abin sk i sign bse nt bse nt Pre se nt bse nt S en sory loss N one ild Pre se nt N one Men in gealsign s bse nt R are bse nt Pre se nt F ever bse nt R are bse nt Pre se nt C erebr spi al F luid P rotein level N ormal ih N ormalor hih ih W h ite cellcoun t Variabl (perm m Tim e to recovery hr af tr tic k W e ks to months Variabl d e pe nd ing on onths to ye ars or no re c ovry (pe rmane nt re moval c ause pare sis) A d aptd f rom lzM W, S mith C S wif tT R: sixye arold irlwith tick paralysis. Up to 5% of children have spina bifida occulta, an incomplete fusion of the posterior vertebral arches, which is usually noted as an incidental radiographic finding. Open neural tube defects (myelomeningocele or spina bifida and anencephaly) usually have multifactorial causes, but in some cases, they may be the result of mendelian recessive inheritance. Daily folic acid intake of 400 mcg reduces the risk of spina bifida by as much as 70%. Serum and amniotic a-fetoprotein can detect open neural tube defects, and ultrasound is also a useful tool. Cerebellar elongation and protrusion of the foramen magnum into the cervical spinal cord. Anatomic anomalies of the hindbrain and skeletal structure result in different positioning of the various structures relative to the upper cervical canal and foramen magnum with different clinical features. Type I is clinically the least severe and is generally asymptomatic during childhood. There may be paroxysmal vertigo, drop attacks, vague dizziness, and headache, which may be increased by the Valsalva maneuver. Occipital headache precipitated by exertion may progress to torticollis, downgaze nystagmus, periodic nystagmus, and oscillopsia. Medulla and cerebellum, together with part or the entire fourth ventricle, are displaced into the spinal canal. This type is strongly associated with noncommunicating hydrocephalus and lumbosacral myelomeningocele. Children with myelomeningocele have a complex, multifaceted, congenital disorder of structure that represents a dysraphic state. In its full expression, it is typified anatomically by the following: n the presence of unfused or excessively separated vertebral arches of the bony spine (spina bifida) n Cystic dilation of the meninges that surround the spinal cord (meningocele) n Cystic dilation of the spinal cord itself (myelocele) n Hydrocephalus and the spectrum of congenital cerebral abnormalities 228. What is the likelihood that a patient with myelomeningocele will have hydrocephalus Hydrocephalus is seen in 95% of children with thoracic or high lumbar myelomeningocele. The incidence decreases progressively with more caudal spinal defects to a minimum of 60% if the myelomeningocele is located in the sacrum. The stridor is usually caused by dysfunction of the vagus nerve, which innervates the muscles of the vocal cords. In their resting position, the edges of the cords meet in the midline; during speech, they move apart. Hence, in bilateral vagal nerve palsies, the free edges of the vocal cords are closely opposed and obstruct air flow, thereby resulting in stridor. In symptomatic patients, the motor nucleus of the vagus nerve may be congenitally hypoplastic or aplastic. More commonly, the vagal dysfunction is believed to arise from a mechanical traction injury caused by hydrocephalus, which produces progressive herniation and inferior displacement of the abnormal hindbrain. Sometimes the later recurrence of stridor indicates the reaccumulation of hydrocephalus as a result of ventriculoperitoneal shunt failure. What are the principal options for managing urinary incontinence in patients with myelomeningocele About 80% of patients have a neurogenic bladder, which most commonly manifests as a small, poorly compliant bladder and an open and fixed sphincter. Hydrocephalus per se does not cause the mental retardation that is associated with this syndrome. In an infant born with myelomeningocele, how does the initial evaluation predict long-term ambulation potential Almost no younger children will ambulate, and only about one third of adolescents will ambulate with the aid of extensive braces and crutches. About one third of children and adolescents will ambulate, but only with extensive assistive devices. The percentage of those able to ambulate is midway between those with high and low lumbar lesions. Nearly half of younger children and nearly all adolescents will ambulate, with varying degrees of braces or crutches. Nearly all children and adolescents will ambulate, with minimal or no assistive devices. What was the first cytotoxic chemotherapeutic agent used for the treatment of children with leukemia In 1948, Sidney Farber reported success using aminopterin (4-aminopteroyl-glutamic acid) in 16 children with acute leukemia. Aminopterin was a precursor to the antifolate drug methotrexate, which is commonly used today. Chemotherapeutic drugs are usually classified by their primary site and mechanism of action or source. The most common are the alkylators, antimetabolites, antitumor antibiotics, and plant toxins. In 1953, investigators discovered that whole guinea pig serum could bring about regression of certain transplantedlymphosarcomas in inbred mice. By 1961, it was determined that the fraction of guinea pig serum responsible for its antileukemic effect contained significant asparaginase activity. Most leukemic lymphoblasts were then found to be asparagine autotrophs, requiring exogenous asparagine for survival. Adjuvant chemotherapy is administered after the primary treatment of a tumor (surgical resection or radiation therapy), when there is no remaining gross tumor that can be assessed for response to the chemotherapy. Neoadjuvant chemotherapy is administered before the delivery of definitive local treatment and then continues afterward in the adjuvant setting. For children with solid tumors, several cycles of neoadjuvant chemotherapy are often administered to improve the chances of achieving complete surgical resection and improved local control of a primary tumor. Which chemotherapeutic agents can be administered intrathecally to either treat or prevent meningeal malignancy Methotrexate, cytarabine, and hydrocortisone are commonly administered intrathecally to treat or prevent meningeal leukemia and lymphoma. It is the study of how drugs are absorbed, distributed, metabolized, and eliminated from the body. Common parameters include elimination half-life, peak concentration, clearance, and area under the concentration-time curve. A pharmacodynamic effect can be a toxicity measurement (decrease in blood counts) or an anticancer measurement (decrease in the size of a tumor) after chemotherapy. This phase is designed primarily to recommend a dose for further testing in children, usually the maximal tolerated dose. Pharmacokinetic studies are performed during phase I trials to help learn whether children handle a drug differently than adults. Usually a group of children with the same diagnosis are studied, and the percentage of patients in whom the drug causes a tumor to decrease in size is determined. Agents include the nitrogen mustards, oxazaphosphorines (including cyclophosphamide and ifosfamide), busulfan, and cisplatin. In high doses, the drug can be nephrotoxic and cause dermatitis, hepatitis, and mucositis. If one had to choose a single laboratory test to obtain before administering high-dose methotrexate, which one should it be Determination of serum creatinine is essential before administering high-dose methotrexate. In the presence of abnormal renal function, high-dose methotrexate carries a high risk for severe or fatal toxicity. Cisplatin causes only mild myelosuppression but is associated with significant nephrotoxicity, ototoxicity, and neurotoxicity. What factors are associated with an increased risk for developing anthracycline-induced cardiotoxicity Total cumulative dose, mediastinal radiotherapy, young age, and female gender are associated with an increased risk for developing anthracycline (doxorubicin, daunorubicin)induced cardiotoxicity. Cumulative anthracycline dose has long been associated with an increased risk, with the incidence of clinically apparent congestive heart failure rising significantly with doxorubicin doses exceeding 450 mg/m2. Late cardiotoxicity appears to be more common in children than in adults because the heart is to unable to grow in proportion to the child, resulting in a small, poorly compliant left ventricle. There is also some evidence that girls have a higher incidence of abnormal cardiac findings at any given cumulative dose than boys. A vesicant is an agent that produces a vesicle; in oncology, it is a chemotherapeutic drug that can cause a severe burn if the drug infiltrates around the intravenous catheter. The anthracyclines (doxorubicin, daunorubicin), dactinomycin, and the vinca alkaloids (vincristine, vinblastine) are all vesicants. These drugs must be administered either through a central venous catheter or through a newly placed, free-flowing intravenous catheter that does not cross over a joint space. Which classes of chemotherapeutic agents have most commonly been implicated in causing secondary leukemias Etoposide-induced leukemias tend to occur earlier, usually within 2 to 3 years of exposure. Why is intrathecal chemotherapy dosed based on patient age, whereas systemic (oral, intravenous) dosing is based on weight or body surface area The brains of children grow disproportionately more quickly than their bodies (hence the tendency of infants who have recently learned to sit to readily tip over). What are the most effective antiemetics for the prevention and treatment of chemotherapy-induced vomiting The serotonin receptor antagonists ondansetron and granisetron are the most effective agents for chemotherapy-associated emesis. They work less well for delayed emesis, for which combinations of antihistamines and phenothiazines may be used. Dexamethasone is a useful adjunct when administering highly emetogenic chemotherapy. The remedy used by the ladies in Arsenic and Old Lace is making an encore performance. In the early 1990s, investigators in China reported that arsenic, an ancient remedy, was found to be highly effective in the treatment of patients with acute promyelocytic leukemia. Arsenic appears to trigger an apoptotic response in promyeloblasts, but its precise mechanism of action is still under investigation. Children who develop the somnolence syndrome have lethargy, headache, and anorexia that last for about 2 weeks. The use of steroids during irradiation appears to minimize the occurrence of the syndrome. This differs from conventional radiation, in which each field has a constant, or fixed intensity profile across the field area and thus allows for dose reduction to normal tissues or critical structures. Because of the physical properties of protons and their ability to deposit energy over a short distance, proton therapy may have the advantage of reducing radiation dose to nontarget normal tissues while allowing higher doses to be delivered to the tumor. Radiation recall is a delayed effect that results from the interaction of certain chemotherapeutic agents (doxorubicin, daunorubicin, or actinomycin-D) with radiation.

Syndromes
- Eye infections
- Medicines to take by mouth
- Weight loss
- Craving and overdose
- Is your child taking any medications? How long has the child taken them?
- West Nile virus
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Alpha-l-antitrypsin deficiency is an autosomal recessive disorder characterized by production of defective alpha-l-antitrypsin infection 6 months after c section 960 mg bactrim sale, which accumulates in hepatocytes and causes liver damage and low serum levels of alpha-l-antitrypsin antibiotic cephalexin buy bactrim 960mg. Reye syndrome is a potentially fatal disease that occurs in young children with viral illnesses treated with aspirin antibiotics that start with z order bactrim with visa. Budd-Chiari syndrome is occlusion of the hepatic vein by a thrombus bacteria animation bactrim 960 mg fast delivery, often resulting in death treatment for dogs with food poisoning order bactrim 960 mg fast delivery. Chronic passive congestion of the liver is a "backup of blood" into the liver augmentin antibiotic 625mg order bactrim once a day, usually due to right-sided heart failure, and may, in long-standing cases, lead to cirrhosis. Malignant tumors include hepatocellular carcinoma, cholangiocarcinoma, angiosarcoma, and metastatic tumors. A 28-year-old intravenous drug user comes to the physician because of a l-week history of weakness, fatigue, and extreme nausea. He says that he typically uses his own needles to inject drugs, but "money was tight" four months ago, and he had been "forced" to share needles with friends. Physical examina tion shows scleral icterus, jaundiced skin, and right upper quadrant abdominal tenderness. Laboratory studies show markedly elevated levels of alanine aminotransferase and aspartate aminotransferase. Which of the following markers is most likely to be positive at this stage of the disease An 18-year-old boy is brought to the physician by his mother because of "uncontrollable shaking" of his right hand. Ring sideroblasts have iron trapped abnormally in mitochondria, forming a ring around nucleus. Signs of anemia include palpitations, dizziness, angina, pallor of skin and nails, content due to the low level of weakness, claudication, fatigue, and lethargy. Reticulocyte index: corrected reticulocyte count/2 Use if bone marrow reticulocytes (shift cells) are present (polychromasia) Divide by 2 because shift cells take twice as long as reticulocytes to mature (2 days versus 1 day). Functional iron is found in hemoglobin, myoglobin, and enzymes (catalase and cytochromes) 11. First is decreased storage iron, which produces reactant and may be artificially elevated in inflammatory Decreased serum ferritin states. Hb H disease: three deletions Number of a genes: 1 (-t a), which produces 25% a chains Increased Hb H (~4, ) forms Heinz bodies, which can be seen with crystal blue stain v. They are expressed postnatally only (therefore only postnatal disease and not prenatal) Ill. Mechanism: mainly due to point mutations, which form either some ~ chains (~+) or none (~O) b. Erythroid hyperplasia in th~ bone marrow causes "crewcut" skull x-ray and increased size of maxilla ("chipmunk face") v. Hemoglobin electrophoresis: t hemoglobin F (90%), t hemoglobin A2, t hemoglobin A 6. May be either pyridoxine (vitamin B6) responsive or pyridoxine unresponsive; the latter is a form of myelodysplastic syndrome (refractory anemia with ring sidero blasts) c. If the patient survives, the resulting hemodilution caused by shift of water from the interstitium will lower the hematocrit c. Release of hemoglobin into the blood causes hemoglobinemia and hemo-glo binuria 11. Markedly decreased hemoglobin-binding proteins in the blood, such as hapto globin and hemopexin, are characteristic v. Abnormality: single nucleotide change in codon causes valine (neutral) to replace normal glutamic acid (acidic) at the sixth position of the ~-globin chain. Increased concentration (dehydration) makes symptoms worse; decreased concentration (with thalassemia) makes symptoms better Parvovirus 11. Capillary thrombi result from sickle cells blocking small vessels and may cause I. Risk of aplastic crisis (especially with parvovirus B19 infection) takes advantage of the differences in pi values vii. Abnormality: single nucleotide change in a codon causes lysine (basic) to replace HbS normal glutamic acid (acidic) at the beta 6 position. Mediterranean type breaking down hydrogen Associated with favism due to ingestion of fava beans peroxide. Heinz bodies cannot be seen with normal peripheral blood stains (Wright Giemsa) 11. Need supravital stains (methylene blue and crystal violet) to see Heinz bodies 111. Heinz bodies are "eaten" by splenic macrophages (extravascular hemolysis), which may form bite cells 6. Chronic hemolysis produces increased bilirubin and an increased risk for jaun dice and pigment gallstones 111. All cells in blood have increased sensitivity to the lytic actions of complement b. Complications: increased risk for aplastic anemia, leukemia, and venous thrombo sis g. Decreased absorption, which may be caused by any of the following: pancreatic proteases. Decreased absorption: intestinal malabsorption (folate is absorbed in the upper small intestine) lll. Decreased utilization: folate antagonists used in chemotherapy such as metho trexate b. Treatment: folate Chapter Summary Red blood cells can have a variety of abnormal shapes or contain inclusions, either of which may suggest particular diagnoses. Anemia is the reduction below normal limits of the total circulating red cell mass, which may lead to palpitations, dizziness, angina, skin pallor, weakness or other symptoms. They can also be classified based on pathogenesis, including broad categories of blood loss, hemolytic anemias, anemias of diminished erythropoiesis. Iron deficiency anemia is a microcytic anemia seen most often in the elderly and poor populations, children, pregnant women, and patients with chronic blood loss. Thalassemias are anemias due to quantitative abnormalities of synthesis of hemoglobin chains, and are subclassified as alpha thalassemias and beta thalassemias. Alpha thalassemia has four clinical forms depending upon the number of alpha-globin genes affected: silent carrier, alpha-thalassemia trait, HbH disease, and hydrops fetalis. Beta thalassemia has three clinical presentations: beta-thalassemia minor, beta-thalassemia intermedia, and beta thalassemia major. Anemia of blood loss occurs when a patient survives acute blood loss and undergoes hemodilution that lowers the hematocrit. Sickle cell anemia is due to a single nucleotide change in the beta-globin chain, and is an important disease of African Americans, it clinically presents as either sickle cell trait or sickle cell anemia. Patients with sickle cell anemia are vulnerable to a variety of complications related to sickled cells blocking small blood vessels. Hemoglobin C disease is also related to a single nucleotide change in a globin gene but produces milder disease than sickle cell anemia. Glucose-6-phosphate dehydrogenase deficiency is an enzyme deficiency that causes red cells to lyse under oxidant stresses. Hereditary spherocytosis is an autosomal dominant disorder due to an abnormal membrane associated protein, spectrin, which leads to spherical erythrocyte morphology with mild to moderate hemolytic anemia. Autoimmune hemolytic anemias can be idiopathic or related to other autoimmune diseases, leukemias and lymphomas, or medications. Paroxysmal nocturnal hemoglobinuria produces episodic hemolysis as a result of increased red-cell sensitivity to the lytic actions of complement. He also says that he occasionally gets a "strange" sensation, as if his heart is "leaping out" of his chest. A 14-year-old girl is brought to the emergency department by a camp counselor because of severe leg pain. She knows that she has a "chronic disease, " but she is not exactly sure what it is. Physical examination shows a 2-cm ulcer on her lower leg that appears to extend to the bone. Infectious mononucleosis (1M) is an example of a virus disease causing lympho cytosis 1. Antibody production: heterophil antibodies (antibodies against other species such as red cells of sheep and horses) are the basis of the Paul-Bunnell reaction used as the monospot test (may be negative first week, so need to repeat test). Clinical infectious monocytosis Age groups include adolescents and young adults ("kissing disease") Symptoms (classic triad): fever, sore throat (see gray-white membrane on tonsils), and lymphadenitis (posterior auricular nodes); fourth sign is hepa tosplenomegaly Mono is an acute, self-limited disease that usually resolves in 4-6 weeks v. Complications include hepatic dysfunction, splenic rupture, and rash if treated with ampicillin Germinal center of follicle Cortex (clones dividing) Paracortex (T-cell rich) Medulla Primary follicle (B-cell rich) Efferent lymphatic Afferent (memory cells exit) lymphatic Hilum (Ag enters) Figure 20-1. Peripheral blood has decreased mature forms and increased immature forms called blasts, which have immature chromatin with nucleoli 11. Bone marrow has increased immature cells (blasts); the diagnostic criteria is >-30% blasts in the bone marrow lll. The Working Formulation divides non-Hodgkin lymphomas into three catego ries based on the prognosis (low-grade, intermediate-grade, and high-grade) Table 20-1. Lymphoblasts are positive for terminal deoxytransferase (TdT) (which is deter testes (Sanduary sites). L2: larger, heterogeneous (pleomorphic) blasts with nuclear clefts malignant cells in brain are Ill. Numerous smudge cells ("parachute cells") are present; smudge cells result from the fact that the neoplastic lymphocytes are unusually fragile f. Lymphocytes have "hairlike" cytoplasmic projections ("dry tap" with bone marrow aspiration) c. Physical exam: a markedly enlarged spleen (splenomegaly) due to infiltrate of red pulp by malignant cells. Chromosome 18 has bcl-2 (activation of bcl-2 inhibits apoptosis by blocking the bax channel) d. Follicular mixed small and large cells (20-50% large cells, which are also called centroblasts, in the follicles) v. Commonly involves the abdomen (such as bowel, retroperitoneum, or ovaries) Burkitt n. Begins as reactive polyclonal reaction and may be associated with previous auto immune disorders d. Twenty percent express Bence-lones proteins, which are light chains that are and Reduced Albumin small and can be filtered into urine c. Lyticbone lesions cause hypercalcemia, bone pain, and increased risk of fracture. Outside bone (extramedullary): usually found within the upper respiratory tract and are not precursor lesions for myeloma g. M protein is found in 1-3% of asymptomatic individuals over the age of 50 (the incidence increases with increasing age) iii. About 20% of these individuals will develop a plasma cell dyscrasia in 10-15 years 9. Russell bodies (cytoplasmic immunoglobulin) and Dutcher bodies (intranuclear immunoglobulin) may be present d. Visual abnormalities due to vascular dilatations and hemorrhages in the retina ii, Neurologic symptoms include headaches and confusion 1p. Clinical symptoms: skin lesions, hypercalcemia, enlarged lymph nodes, liver, and spleen c. Sequence of skin changes (stages): inflammatory eczematous ~ plaque stage ~ tumor nodule stage d. Cerebriform Sezary cells in peripheral blood: Sezary syndrome (which is also asso ciated with a generalized exfoliative skin rash) F. Spread is contiguous to adjacent node groups (unlike non-Hodgkins lympho mas) lll. Mixed cellularity; has eosinophils and plasma cells (increased number of eosinophils is related to lL-5 secretion) iii. B-cell symptoms: fever (that comes and goes = Pel-Ebstein fever), weight loss, night sweats. Survivors of chemotherapy and radiotherapy have increased risk for non Hodgkin lymphoma or acute leukemia Chapter Summary Leukocytosis is a common reactive pattern of white cells; determining whether the leukocytosis is related to neutrophilia, eosinophilia, monocytosis, or lymphocytosis may be helpful in narrowing the diagnostic possibilities. Infectious mononucleosis is a common viral disease typically lasting 4 to 6 weeks that can cause lymphocytosis, fever, sore throat, lymphadenitis, and hepatosplenomegaly. Acute nonspecific lymphadenitis tends to cause tender lymph nodes and can be seen with bacterial or viral infections. Chronic nonspecific lymphadenitis tends to cause non-tender lymph nodes and can be seen with chronic inflammatory conditions, viral infections, medicines, and in nodes draining cancers. Acute leukemias are characterized by more than 30% blasts in the bone marrow, which may also be identified in the peripheral blood. Clinically, acute leukemias cause symptoms related to marrow failure, such as anemia, fatigue, increased infections, fever, and bleeding.
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