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In those identified as carrying the mutant gene medicine to stop diarrhea order donepezil no prescription, performed because of the potential for later developroutine screening for individual manifestations of ment of malignant carcinoid tumors medications 142 order cheap donepezil. Islet cell carcinomas fre601 glands from the neck (leaving ~50 mg of parathyroid quently metastasize to medications osteoporosis order donepezil australia the liver but may grow slowly treatment of hyperkalemia buy 5mg donepezil visa. As this issue arises with some quinagolide) usually returns the serum prolactin level to frequency in the context of parathyroid disease in normal and prevents further tumor growth. Transsphenoidal resection is approand it may be useful prior to the initial operation. First, nist, rapidly lowers insulin-like growth factor levels in the pancreatic islet cell tumors are multicentric, maligpatients with acromegaly. Radiation therapy may be nant about a third of the time, and cause death in useful for large or recurrent tumors. As a tions that include neuropathy, retinopathy, and result, other neoplastic manifestations that develop nephropathy. These features make it difficult to formulater in the course of this disorder, such as carcinoid synlate clear-cut guidelines, but some general concepts drome, are now seen with increased frequency. Hormonal abnormalities can sometimes usually develops in childhood, beginning as hyperplasia be controlled. About half of the the most distinctive features and are recognizable in childtumors are bilateral, and >50% of patients who have had hood. Neuromas are present on the tip of the tongue, unilateral adrenalectomy develop a pheochromocytoma under the eyelids, and throughout the gastrointestinal tract in the contralateral gland within a decade. The ture of these tumors is a disproportionate increase in the most common presentation in children relates to gastroinsecretion of epinephrine relative to norepinephrine. Each germ-line mutation changes a cysteine at thyroid carcinoma; Signal, the signal peptide; Cadherin, a codons 609, 611, 618, 620, or 634 to another amino acid. With greater mutations in codons 630, 768, and 804 have also been experience, mutations that were once associated with identified (Fig. Establishment of the specific germ-line other malignancies where germ-line mutations of cancermutation facilitates the subsequent analysis of other famcausing genes contribute to a greater percentage of ily members. Each family member at risk should be tested apparently sporadic cancer than previously considered. Individuals in a kindred with a known mutation who cium testing should be performed on members at risk. In children with codon 611, 618, 620, 630, 634, and clinical experience is insufficient to exclude pheochro891 mutations, thyroidectomy should be performed mocytoma screening in these individuals. Finally, there are for individuals with abnormal screening tests or with kindreds with codon 609, 768, 790, 791, 804, and 912 symptoms suggestive of pheochromocytoma (Chap. The clinician caring for children with one undetected pheochromocytoma can cause maternal of these mutations faces a dilemma. However, in other kindreds there are mone levels every 23 years provides an adequate examples of metastatic disease occurring early in life. For screen for hyperparathyroidism, except in those families example, metastatic disease prior to the age of 6 years in which hyperparathyroidism is a prominent compohas been described with codon 609 and 804 mutations nent, where measurements should be made annually. Total thyroidectomy form a total thyroidectomy with or without central node with a central lymph node dissection should be perdissection at some arbitrary age (perhaps 610 years of formed in children who carry the mutant gene. Incomage), or (2) continue annual or biannual calcitonin plete thyroidectomy leaves the possibility of later provocative testing with performance of total thyroidectransformation of residual C cells. The goal of early thertomy with or without central neck dissection when the apy is cure, and a strategy that does not accomplish this test becomes abnormal. Long-term follow-up studies indimeasurement of serum calcitonin basally and 2, 5, 10, cate an excellent outcome, with ~90% of children free of and 15 min after a bolus injection of 5 g pentagastrin disease 1520 years after surgery. Patients should be warned of patients in whom the diagnosis is made on the basis before pentagastrin injection of epigastric tightness, of a palpable thyroid nodule die from the disease within nausea, warmth, and tingling of extremities and reas1520 years. Chemotherapy with combinations of adricodon 883, 918, and 922 mutations, especially in newamycin, vincristine, cyclophosphamide, and dacarbazine born children where the diagnosis is suspected but the may provide palliation. Endocrine tumors also form in less common developing pheochromocytoma in the apparently unafneoplastic genetic syndromes. These include Cowdens fected gland over an 8to 10-year period, and the risks disease, Carneys complex, familial acromegaly, and of adrenal insufficiency caused by removal of both familial carcinoid syndrome. Most clinicians nerve schwannomas; spotty skin pigmentation; and tesrecommend removing only the affected gland. However, life and requires two of three components for diagnosis: many patients with antimicrosomal and antithyroglobulin mucocutaneous candidiasis, hypoparathyroidism, and antibodies never develop abnormalities of thyroid funcadrenal insufficiency. Thus increased antibody titers alone are poor prehypoparathyroidism present with similar high frequency dictors of future disease. Adrenal insufficiency include hypophysitis, celiac disease (23%), atrophic gasis observed in 6072% of patients. Vitiligo, caused by and glucocorticoids may be lost simultaneously or antibodies against the melanocyte, and alopecia are less sequentially. A few patients develop a lateOther endocrine defects can include gonadal failure onset, usually transient hypoparathyroidism caused by (60% female, 14% male), hypothyroidism (5%), and antibodies that compete with parathyroid hormone for destruction of the beta cells of the pancreatic islets and binding to the parathyroid hormone receptor. Up to development of insulin-dependent (type 1) diabetes 25% of patients with myasthenia gravis, and an even mellitus (14% lifetime risk). The roles Consequently, such patients should be screened for of cytokines such as interferon and cell-mediated immuadrenal disease. Patients with insulin receptor antibodies and acanthoScreening measurements of autoantibodies against potensis nigricans are often middle-aged women who acquire tially affected endocrine organs are of uncertain prognosinsulin resistance in association with other autoimmune tic value. Vitiligo, alopecia, Raynauds phenomenon, roidism due to glandular agenesis and mucocutaneous and arthritis may also be seen. Other autoimmune candidiasis), Kearns-Sayre syndrome (hypoparathyendocrine disorders, including thyrotoxicosis, hypothyroidism, primary hypogonadism, type 1 diabetes mellitus, roidism, and hypogonadism, occur rarely. Others have severe diabetes mellitus ease involves hormone replacement and is covered in requiring massive doses of insulin (several thousand units detail in the chapter on thyroid disease (Chap. Prithe antibodies determines the manifestations; although mary hypothyroidism can mask adrenal insufficiency insulin resistance is more common, fasting hypoglycemia by prolonging the half-life of cortisol; consequently, can result from insulinomimetic antibodies. This disorder cocorticoids prior to or concurrently with thyroid horis characterized by ataxia, telangiectasia, immune abnormone therapy. Hypoglycemia or decreasing insulin malities, and an increased incidence of malignancies. The endocrine manifestations suggest an autoimmune basis of the disorthis disorder typically occurs in patients with other der, but circulating antibodies against endocrine cells have autoimmune disorders and is caused by polyclonal not been demonstrated. Increased serum and tissue levels autoantibodies that bind to endogenously synthesized of interleukin 6, interleukin 1, vascular endothelial insulin. If the insulin dissociates from the antibodies sevgrowth factor, matrix metalloproteins, and tumor necrosis eral hours or more after a meal, hypoglycemia can result. In plasma plasma cell dyscrasia such as local radiation of bony lesions, cell dyscrasias such as multiple myeloma, the plasma cells chemotherapy, thalidomide, plasmapheresis, bone marrow or may produce monoclonal antibodies against insulin and stem cell transplantation, and treatment with all-trans retinoic cause hypoglycemia by a similar mechanism. Ann Intern Med hormone immunoassays and cause false elevations or 146:425, 2007 decreases in measured hormone levels. J Clin Endocrinol Metab women and testicular failure, impotence, and gyneco92:1948, 2007 mastia in men. These vascularized tumors that arise from cells derived from the tumors may arise sporadically or be inherited as feasympathetic. The the name pheochromocytoma refiects the black-colored diagnosis of pheochromocytomas provides a potenstaining caused by chromaffin oxidation of catecholamines. The clinical presentation is variable, ranging pheochromocytoma to describe symptomatic catecholaminefrom an adrenal incidentaloma to a patient in hyperproducing tumors, including those located in extraadrenal tensive crisis with associated cerebrovascular or cardiac retroperitoneal, pelvic, and thoracic sites. Classically, patients components involved in cell cycle progression, glucose have episodic hypertension, but sustained hypertension is metabolism, and oxygen sensing. During episodes of hormone release, which can occur at very divergent intervals, patients are anxious and the clinical presentation is so variable that pheochropale, and they experience tachycardia and palpitations. Among the presenting symptoms, episodes be precipitated by surgery, positional changes, exercise, of palpitations, headaches, and profuse sweating are typipregnancy, urination (particularly bladder pheochromocycal and constitute a classic triad. Both are of equal Headaches Weight loss importance, although measurement of catecholamines is Sweating attacks Paradoxical response to traditionally the first step. Palpitation and tachycardia antihypertensive drugs Hypertension, sustained or Polyuria and polydipsia paroxysmal Constipation Biochemical Testing Anxiety and panic attacks Orthostatic hypotension Pheochromocytomas and paragangliomas synthesize and Pallor Dilated cardiomyopathy Nausea Erythrocytosis store catecholamines, which include norepinephrine (noraAbdominal pain Elevated blood sugar drenaline), epinephrine (adrenaline), and dopamine. Elevated Weakness Hypercalcemia plasma and urinary levels of catecholamines and the methylated metabolites, metanephrines, are the cornerstone for the diagnosis.

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At the age of 2 y 1m xanax medications for anxiety buy donepezil overnight delivery, she was develop even at normal or mildly elevated blood pressure levels hospitalized with signs of respiratory failure treatment hyperthyroidism cheap donepezil 5mg line. The resection of 220cm of intestine 1 Department of Pediatrics medicine you can give cats buy donepezil overnight delivery, University Hospital of Poitiers medications given for uti generic donepezil 5mg overnight delivery, Poitiers was performed, a looped ejunostomy was bred and a direct anastomosis France, 2 Department of Pediatrics, University Hospital of Rennes, was formed. United Kingdom Results: Twenty-nine patients have been already included and around 60 patients are expected. Three patients had a proMethods: We searched Medline, Embase, PubMed, Web of teinuria at 15 years old. Overall, high levels of heterogeneity were 1Ankara University Medical Faculty, Department of Pediatrics, Division seen between studies. The aim of this study was to evaluate the though this was consistent with chance (p=0. Females are more likely to present late, followed in our out-patient clinic between 2014-2019 were retrowhich confirms single-study findings. Patient data including blood pressure measureimportant to further our understanding of this problem and local ments, end-organ damage and treatment adherence were recorded populations should identify barriers beyond gender and disease to from hospital records at diagnosis and in the following 1, 2, 3 improve access to care. On admission, left ventricular mass index was pura is mostly dependant on the severity of renal involvement. Some medical centers thy was detected in 9 patients and all ameliorated within 5 years. EndMaterial and methods: this was a multicenter, retrospective, organ damage was improved with medication. The target population was comprised of patients did not lose weight and they were still hypertensive. Some of the patients had an early renal biopsy (before starting treatment or in the folS. However, this is a small patient cohort and Analyzed age, gender, weight, etiology of renal disease, positive there is missing data. Results: Detected significantly higher mortality in patients when the etiology of kidney disease was of cardiac origin 10/20 (50%), H. We did not find significant differences regarding the loaded with tacrolimus in our centre. Data presented as Conclusions: the mortality was directly associated with the etiolomedian. Statistical testing using independent samples Manngy of renal disease, with the low age and the lower weight of the Whitney U test for non-parametric data. Therefore, when we group A 2 patients (1 white, 1 black) and group B 3 patients(1 white, 1 black, identify a patient with refractory edema, alternative therapies should be 1 Asian) did not reach target levels within 2 weeks(all sub-therapeutic). The overfill theory explains the nephrotic syndrome edema Asides from nausea in 1 patient no other adverse effects noted due to preindependent of the renin-angiotensin-aldosterone system. By days 2-7 and week 2 the pre-loaded group suffering from cortico-resistant nephrotic syndrome for 6 months. It presents significant edema (six kilos of edema and a dry weight of ten would be helpful to determine if this had an impact on graft function as kilos), refractory to initial therapy (intravenous albumin, furosemide and well as carrying out this analysis in a larger cohort. Rojo5, the proteinuria (protein / creatinine ratio from isolated urine sample 70). Peredo After ten days of therapy, the patient developed significant hyponatremia, 9,P. Conclusion: this case report reinforces the overfill theory of nephrotic Gustavo Fricke, Vina del Mar Chile, 3Clinica Davila, Santiago de Chile syndrome edema. Amiloride in combination with Hydrochlorothiazide has Chile, 4 Hospital Luis Calvo Mackenna, Santiago de Chile Chile, shown to be effective in the management of refractory edema in a pediatric 5 Hospital Exequiel Gonzalez Cortes, Santiago de Chile Chile, patient. However, the possibility of hyponatremia as a side effect should be 6 Hospital Exequiel Gonzalez Cortes Santiago de Chile Chile, evaluated, especially if Amiloride is combined with Hydrochlorothiazide. A descriptive, retrospective, tion of perinatal asphyxia remains underdiagnosed in our care observational study of clinical and laboratory data was done using inforsetting. The purpose of this study was to determine the prevalence mation registered in the database of Paediatric Nephrology Branch of of renal impairment during perinatal asphyxia in term newborns Chilean Paediatrics Society in 2017, with patients on peritoneal dialysis. The mean estimated Glomerular Filtration Rate with perinatal asphyxia from February 1st to July 31st, 2016 at university was 10,3 ml/min/1. We bemanagement of neonates with perinatal asphyxia should take into considlieve this could be due to the heterogeneous phosphorus chelations, diferation multisystem disorders. The microorganisms recovered from blood cultures Foundation Trust, London United Kingdom were: Staphylococcus aureus (45. Office of National Statistics data used to calculate standardised mortality ratio. Additional data collected Material and methods: this was a retrospective (2-year), singlesuggests multi-factorial deathswhich may not be captured with traditional center study (Femme-Mere-Enfant hospital of Lyon in France) of data fields. The correlation between proteinemia and albuminemia, by the Pearson method, was evaluated in children D. Despite pediatric hemodialysis has dosages) and idiopathic in the other 92 (424 dosages). The corprogressed, the vascular access remains a crucial factor in the relation between albumin and proteinemia with the Pearson methcontinuity of treatment. This suggests that one could do without the results of the children, which is associated with an increased risk of infection. A descriptive, retrospective, observational children with kidney cysts indicates the early damage to the renal parenstudy of clinical and laboratory data was done using information chyma, detectable before the onset of hypertension and other organs registered in the database of Paediatric Nephrology Branch of damage. We believe this could be due to several factors seizure, demonstrating a sodium of 119mmol/l. Plasma sodium remained refractory(1161 Cardiological Outpatient Medicor, Myszkow Poland, 2 Department of 122mmol/l) and nephrology input sought. Serum osmolality Pediatrics, School of Medicine with the Division of Dentistry in Zabrze, 250mosml/l, urinary osmolality 520mosml/l, urinary sodium Medical University of Silesia in Katowice Poland, 3 Chair and 171mmol/l. Katowice Poland, 4 Pediatric Nephrology Ward, Public Clinical Paired plasma/urine tests were: sodium 119/137mmol/l, creatinine Hospital No. Fractional excretion of sodium Developmental Defects Surgery and Traumatology, School of Medicine 1%. Familial testing showed his mother and maternal vided into genetic and acquired cysts. It is clinically important to idenmaintaining a plasma sodium above 130mmol/l and had no further tify markers of early kidney damage. Pediatr Nephrol (2019) 34:18212260 2165 We suspect this boy had chronic asymptomatic hyponatremia, & After implementation of Protocol 2,mean values measured were Hb: acutely exacerbated by the elimination of dairy and timing of full 116. Protocol 2 maintained haemoglobin levels within a more satisfactory range than Protocol 1. Keogh 1, Background: Renal anaemia is an ongoing challenge in paediatric 2 1 1 1 1 F. Abdelrahman 2 1Cairo University Egypt, 2 Department of pediatrics, Kasr Al Ainy School of Medicine, Cairo University Egypt & Protocol 1 mean values: Hb: 114. Protocol 2 Introduction: Kidney transplantation has been established as the was devised with a higher acceptable minimal haemoglobin most efficient treatment of end stage renal disease with the advanparameter. In the on regular follow up in renal Transplantation clinic in Abo el Reesh diagnosis and treatment process, the most common complications hospital were enrolled to detailed history and physical examination, labin patients with nephrotic syndrome include infection, electrolyte oratory investigation. An informed written consent was obtained from imbalance, low blood volume, hypercoagulability and thrombosis, parents before enrollment. Patients complicated nephrotic syndrome age ranges between 10 to 15 years than non-obese recipients. Graft with pancreatic lesions are rarely reported, and childrensclinical loss was significantly higher among obese compared with nonmanifestations are very atypical. Obese patients displayed lower survival compared observe clinical manifestations, laboratory tests and related imagwith non-obese subjects at 5 years but statistically not significant ing of children complicated in our hospital, and to explore its (p-value= 0. Conclusion: obesity is an increasing problem in children who presMaterial & methods: We retrospectively analyzed the clinical data ent fpr transplantation which may represent an independent risk of 6 children complicated with Primary nephrotic syndrome and factor for graft loss and patient death. All patients were improved after 1 University of Puerto Rico Puerto Rico, 2 University of Puerto Rico; Octreotide Acetate injection and supportive treatment. Short-term progAuxilio Mutuo Hospital Transplant Center Puerto Rico nosis is good. Department of Nephrology and Rheumatology, Childrens Hospital of Results: From 1977-2013, 146 children, female: 39%, Hispanic Shanghai, Shanghai Jiaotong University, Shanghai China (Caribbean): 100%, age: 14yrs (2-18yrs) were transplanted.

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Precipitate on the film Causes: unclean slides treatment 7th march order donepezil 10 mg on-line, drying during the period of staining medicine glossary donepezil 10 mg discount, inadequate washing of slide at the end of the staining period Correction: use clean slides symptoms multiple sclerosis order donepezil cheap online, cover the smear with generous amount of the stain treatment gastritis buy generic donepezil line, wash the slide until thinner parts of the film are pinkish 84 Hematology Review Questions 1. Introduction Visual counting of blood cells is an acceptable 86 Hematology alternative to electronic counting for white cell and platelet counts. The main principles for such examinations are: Selection of a diluting fluid that not only will dilute the cells to manageable levels but will either identify them in some fashion or destroy contaminant cellular elements. The ruled area itself is divided by microscopic lines into a pattern that varies again with the type of the chamber. The 4 corner squares are divided into 16 squares, each with an area of 1/16 of a mm2. The central ruled area of 1mm2 is divided into 16 large squares by sets of triple lines. The Improved Neubauer Counting Chamber the depth between the lower surface of the cover glass which is on the raised bars and the ruled area is 0. These squares are subdivided to form 16 smaller squares, each with an area of 1/16 of 1mm2 (figure 6. Burker ruled counting chamber Like the Neubauer counting chamber, this has a ruled area of 9mm2 and a depth of 0. Dilution of the Sample Dilution of sample is accomplished by using either a thomma pipette or the tube dilution method. Thomma pipettes are small calibrated diluting pipettes designed for either white cell or red cell count. Principle Whole blood is diluted 1 in 20 an acid reagent which hemolyzes the red cells (not the nucleus of nucleated red cells), leaving the whit cells to be counted. The glacial acetic acid causes erythrocyte lysis while the gentian violet lightly stains the leucocytes permitting easier enumeration. Test method Thomma White Cell Pipette the long stem is divided into 10 equal parts with 0. Once the pipette accurately filled to the mark, the rubber suction (or mouth piece) is carefully removed, with the pipette held horizontally and only one finger sealing the tip. Both ends of the pipette may then be sealed with special small rubber sealing caps or with the middle finger on the tip and the thumb on the other end. The cover glass is placed on the chamber and a slight pressure applied to the ends of the cover glass until a 96 Hematology rain bow or Newtons diffraction rings are revealed on either side. Once the diluted blood in the pipette has been thoroughly mixed, a few drops are expelled to discard the cell-free diluting fluid in the long stem of the pipette. With the index finger forming a controlled seal over the end of the pipette, which is held at an angle of 450, the tip of the pipette is brought up to the edge of the cover glass and by gentle release of index finger pressure, fluid is allowed to run out slowly until the counting platform is covered. The chamber is placed in position on the microscope stage and is allowed to stand for 2 or 3 minutes so that the cells will settle. Pipettes (thomma and sahli) should be washed well with a sequence of water and acetone (filled with 97 Hematology each fluid three or four times) and air drawn after the acetone until the inside of the pipette is thoroughly dry. Pipettes should be periodically cleaned with potassium dichromate cleaning solution or hydrogen peroxide. Hemocytometers should be washed in distilled water immediately after use and dried with gauze or tissue paper. The corrected leucocyte count Nucleated red cells will be counted and can not be distinguished from leucocytes in the total leucocyte count. Using a capillary, Pasteur pipette, or plastic bulb pipette held at an angle of about 450C, fill one of the grids of the chamber with the sample, taking care not to overfill the area. Leave the chamber undisturbed for 2 minutes to allow time for the white cells to settle. Count as described in thomma white cell count method * When a count is higher than 50 x 109/l, repeat the count using 0. Total leucocyte counts are commonly increased in infections and when considered along with the differential leucocyte count can be indicators as to whether the infecting agent is bacterial or viral. Red Cell Count Although red cell counts are of diagnostic value in only a minority of patients suffering from blood diseases, the advent of electronic cell counters has enormously increased the practicability of such counts. Their value, too, has been increased now that they can be done with a degree of accuracy and reproducibility comparable to that for hemoglobin estimation. Diluting Fluid 1% formal citrate Dilution Thomma Red Cell Pipette Take a well mixed blood or blood from a freely flowing capillary puncture to the 0. It is important to count as many cells as possible for the accuracy of the count is increased thereby; 500 cells should be considered as the absolute minimum. Platelet counts are also performed when patients are being treated with cytotoxic drugs or other drugs which may cause thrombocytopenia. Method using formal-citrate red cell diluent Diluent should be prepared using thoroughly clean glassware and fresh distilled water. Then fill a Neubauer counting chamber and allow the platelets to settle for 20 minutes. To prevent drying of the fluid, place the chamber in a petri dish or plastic container on dampened tissue or blotting paper and cover with a lid. Method Using Ammonium Oxalate (10g/l; 1%w/v) this diluent causes erythrocyte lysis. The cells are counted in 5 small squares in the central 1mm2 of the improved Neubauer counting chamber. Rough estimation of platelet number from a stained blood film Normally there are 10-20 platelets per oil immersion field. Special care must be taken when counting platelets: To check there are not clots in the blood sample. Principle Blood is diluted with a fluid that causes lysis of erythrocytes and stains eosinophils rendering them readily visible. Diluting Fluid Hinklemans fluid It has the advantage of keeping well at room temperature and not needing filtering before use. Method Make dilution of blood using thomma pipette or tube dilution as described for the white cell count. Reference range 40 440 fi 106/l Interpretation of eosinophil counts Eosinophilia is common in allergic conditions. This should result in a film in which there is some overlap of the red cells diminishing to separation near the tail and in which the white cells on the body of the film are not too badly shrunken. The polymorphonuclear leucocytes and monocytes predominate at the edges while much of smaller lymphocytes are found in the middle. Methods of Counting Various systems of performing the differential count have been advocated. The problem is to overcome the differences in distribution of the various classes of cells which are probably always present to a small extent even in well made films. Of the three methods indicated underneath for doing the differential count, the lateral strip method appears to be the method of choice because it averages out almost all of the disadvantages of the two other methods. Multiple manual registers or 118 Hematology electronic counters are used for the count. The Longitudinal Strip Method the cells are counted using the X40 dry or X100 oil immersion objectives in a strip running the whole length of the film until 100 cells are counted. If all the cells are counted in such a strip, the differential totals will approximate closely to the true differential count. It should be related to the total leucocyte count and the results reported in absolute numbers. If they are included, they are expressed as a percentage of the total nucleated cell count. Myelocytes and metamyelocytes, if present, are recorded separately from neutrophils. An increase may point to an inflammatory process even in the absence of an absolute 122 Hematology leucocytosis. The Cook-Arneth Count Arneth attempted to classify the polymorphonuclear neutrophils into groups according to the number of lobes in the nucleus and also according to the shape of the nucleus. The count is performed by examining 100 neutrophils and placing them in their correct class: Class I: No lobes (An early cell in which the nucleus has not started to lobulate). That means if the figures were to be plotted on graph paper, the peak of the graph would move to the left hand side of the normal curve.

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