By I. Ur-Gosh. Capitol College. 2018.

Clinical syndrome/ Pyridoxine is unusual in that both deficiency and overdose cause neuropathies buy ventolin 100mcg lowest price. Toxicity from high doses causes a sensory neuropathy with prominent sensory ataxia ventolin 100mcg with visa. Pathogenesis How pyridoxine deficiency and overdose cause neuropathy is unclear buy ventolin 100mcg cheap. Defi- ciency results from polynutritional deficiency buy generic ventolin 100mcg on line, chronic alcoholism 100 mcg ventolin mastercard, and from treatment with isoniazid and hydralazine. Isoniazid inhibits conversion of pyridoxine to pyridoxal phosphate. Increased pyridoxine can be detected in the urine, but this is not important for diagnosis. Diagnosis Deficiency can be easily diagnosed by checking blood levels of pyridoxine. EMG shows predominantly sensory abnormality in pyridoxine toxicity, but can show some mild motor involvement as well. Differential diagnosis Pyridoxine deficiency looks like other nutritional and metabolic sensory/motor axonal neuropathies. Therapy 100–1000 mg pyridoxine given daily during isoniazid or hydralazine treatment is effective. Deficiency caused by alcoholism or other states of malnutrition should be treated with pyridoxine and other vitamins, since other deficiencies are likely concurrent. Prognosis The deficiency neuropathy may improve with pyridoxine replacement or when INH is stopped. The sensory neuropathy caused by overdose shows little improvement. References Bernstein AL (1990) Vitamin B6 in clinical neurology. Ann NY Acad Sci 585: 250–260 Snodgrass SR (1992) Vitamin neurotoxicity. Mol Neurobiol 6: 41–73 301 Strachan’s syndrome Genetic testing NCV/EMG Laboratory Imaging Biopsy + Axonal degeneration with myelin breakdown is seen in the posterior columns Anatomy/distribution of the cervical cord and optic nerves. Sural nerve biopsy shows axonopathy of large diameter fibers. Patients report symptoms of sensory neuropathy (painful and burning feet). Symptoms Strachan’s syndrome is defined by painful neuropathy, amblyopia, and orogen- Clinical syndrome/ ital dermatitis. The patients treated with vitamins during the Cuban outbreak responded well, and thus it is thought that the pathology is due to poly- deficiency of thiamine, niacin, riboflavin, and pyridoxine. Multivitamin replacement with a nutritious diet is effective. Replacement of Therapy riboflavin (B2) quickly affects orogenital dermatitis, but has no effect on neuro- logical symptoms. Prognosis Cockerell OC, Ormerod IE (1993) Strachan’s syndrome: variation on a theme. J Neurol Reference 240: 315–318 302 Thiamine neuropathy Genetic testing NCV/EMG Laboratory Imaging Biopsy ++ + ++ Anatomy/distribution Thiamine deficiency causes degeneration of sensory and motor nerves, vagus, recurrent laryngeal nerve, and brainstem nuclei. Lactate accumulates in axons due to the absence of thiamine diphosphate and transketolase. Symptoms The symptoms indicate a sensory and motor neuropathy: distal paresthesias, aches and pains, and limb weakness. Clinical syndrome/ “Dry Beriberi” is characterized by painful distal paresthesias, ankle areflexia, signs and motor weakness. Pathogenesis Beriberi is caused by states of poor nutrition: starvation, alcoholism, excessive and prolonged vomiting, post-gastric stapling, or unbalanced diets of carbo- hydrates without vitamins, protein, or fat (polished, milled rice or ramen noodles). The importance of thiamine to carbohydrate metabolism may be the cause of the nervous system damage.

This change is reversible order ventolin 100 mcg visa, however 100mcg ventolin with mastercard; distal marrow extension can result from intensive stimulation generic ventolin 100 mcg fast delivery, as occurs with severe hemolytic anemias buy generic ventolin 100mcg, long-term administration of hematopoietic growth factors ventolin 100mcg line, and hematologic malignancies. The term medullary hematopoiesis refers to the production of blood cells in the bone marrow; the term extramedullary hematopoiesis indicates blood cell production outside the marrow in the spleen, liver, and other locations. A 35-year-old woman with advanced HIV disease complicated by anemia is seen for routine follow-up. The patient is started on erythropoeitin to decrease the severity of her anemia and to provide sympto- matic improvement. Which of the following laboratory findings is the most easily monitored immediate effect of erythro- poietin therapy? An increase in the mean corpuscular hemoglobin level Key Concept/Objective: To understand that an increase in the reticulocyte count is the most easi- ly monitored immediate effect of erythropoietin therapy 5 HEMATOLOGY 3 Erythropoietin is a glycosylated protein that modulates erythropoiesis by affecting several steps in red cell development. The peritubular interstitial cells located in the inner cortex and outer medulla of the kidney are the primary sites for erythropoietin production. Erythropoietin can be administered intravenously or subcutaneously for the treatment of anemia caused by inadequate endogenous production of erythropoietin. Treatment is maximally effective when the marrow has a generous supply of iron and other nutrients, such as cobalamin and folic acid. For patients with renal failure, who have very low ery- thropoietin levels, the starting dosage is 50 to 100 units subcutaneously three times a week. The most easily monitored immediate effect of increased endogenous or exogenous ery- thropoietin is an increase in the blood reticulocyte count. Normally, as red cell precursors mature, the cells extrude their nucleus at the normal blast stage. The resulting reticulo- cytes, identified by the supravital stain of their residual ribosomes, persist for about 3 days in the marrow and 1 day in the blood. Erythropoietin shortens the transit time through the marrow, leading to an increase in the number and proportion of blood reticulocytes within a few days. In some conditions, particularly chronic inflammatory diseases, the effectiveness of erythropoietin can be predicted from measurement of the serum erythro- poietin level by immunoassay. It may be cost-effective to measure the level before initiat- ing treatment in patients with anemia attributable to suppressed erythropoietin produc- tion, such as patients with HIV infection, cancer, and chronic inflammatory diseases. Several studies have shown that erythropoietin treatment decreases the severity of anemia and improves the quality of life for these patients. In patients with anemia caused by can- cer and cancer chemotherapy, current guidelines recommend erythropoietin treatment if the hemoglobin level is less than 10 g/dl. A 55-year-old man with type 1 diabetes undergoes dialysis three times a week for end-stage renal disease. You recently started him on erythropoietin injections for chronic anemia (hematocrit, 25%). Which of the following is the best test to determine whether this patient will respond to the erythro- poietin treatment? Blood urea nitrogen Key Concept/Objective: To understand the site of production, effect, and therapeutic monitoring of erythropoietin In many renal diseases, the kidneys fail to produce sufficient amounts of erythropoietin. Replacement of endogenous erythropoietin stimulates red cell precursors in the bone mar- row to mature more quickly. If the patient has normal bone marrow, an elevated reticulo- cyte count should be seen several days after initiation of therapy. A 34-year-old woman undergoes chemotherapy for advanced-stage breast cancer. Which cell line would you expect to be the last to recover in this patient? RBCs Key Concept/Objective: To understand the time needed for cell-line recovery after bone marrow damage 4 BOARD REVIEW The proliferation and maturation of platelets take longer than those of either red blood cells (7 to 10 days) or white blood cells (10 to 14 days) and thus are the slowest to recover from an acute bone marrow injury, such as occurs with chemotherapy. An 86-year-old man visits your clinic for routine follow-up. Upon questioning, the patient admits to worsening dyspnea on exertion and generalized fatigue. He denies having fever, chills, cough, dysuria, blood loss, or weight loss. Routine laboratory studies reveal a hemoglobin concentration of 8. The patient denies eating nonfood substances but does admit to craving and eating large amounts of ice daily. The patient’s stool is positive for occult blood by guaiac testing.

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Vastus medialis oblique/vastus lateralis mus- Rehab 2001 order 100mcg ventolin fast delivery; 82: 1692–1695 ventolin 100mcg sale. The knee as a biologic transmission with an and without patellofemoral pain syndrome quality 100mcg ventolin. Clin Orthop Rel Res 1996 buy ventolin 100 mcg without prescription; 325: 1995 cheap 100mcg ventolin mastercard; 75(8): 672–682. The McConnell regimen for anterior knee pain: A randomised controlled trial. Cowan, SM, KL Bennell, PW Hodges, KM Crossley, and drome. Effect of patellar tus medialis obliquus and vastus lateralis in subjects taping on knee kinetics of patients with patellofemoral with patellofemoral pain syndrome. J Orthop Sports Phys Ther 1999; 29(11): Rehabil 2001; 82: 183–189. Awareness of the retinaculum in evaluat- obliquus relative to vastus lateralis in subjects with ing patellofemoral pain. Am J Sports Med 1982; 10(3): patellofemoral pain syndrome. Cowan, SM, PW Hodges, KL Bennell, and KM Patellofemoral Joint, 2nd ed. Altered vastii recruitment when people with Wilkins, 1990. The effect of Arch Phys Med Rehabil 2002; 83: 989–995. Therapeutic patel- and vastus lateralis muscle activity in persons with lar taping changes the timing of vastii muscle activation patellofemoral pain. Cowan, SM, PW Hodges, KL Bennell, KM Crossley, Sports Exer 1994; 26(1): 10–21. A biome- ment of the vastii in untrained postural tasks can be chanical and clinical evaluation of a patellofemoral restored by specific training. Patellar taping: as an intervention for patellofemoral pain. J Orthopaed Is clinical success supported by scientific evidence? Crossley, K, K Bennell, S Green, S Cowan, and on perceived pain and knee extensor torques during J McConnell. Conservative management of isokinetic exercise performed by patients with patellofemoral pain: A randomised, double-blind con- patellofemoral pain. Analysis domized controlled trial of physical therapy treatment of outcome measures for persons with patellofemoral programs in patellofemoral pain syndrome. Physioth pain: Which outcome measures for individuals with Can Spring 1999; 93–106. Heintjes, E, MY Berger, SM Bierma-Zeinstra, RM Med Rehabil 2004; 85: 815–822. Crossley, KM, SM Cowan, KL Bennell, and J McConnell. Cochrane Database Knee flexion during stair ambulation is altered in indi- of Systematic Reviews 2003; CD003472. In oblique activing in the presence of patellofemoral pain. Conservative Management of Anterior Knee Pain: The McConnell Program 183 41. Maximum bilateral contrac- rupted during eccentric contractions in subjects with tions are modified by neurally mediated interlimb patellofemoral pain. Ireland, ML, JD Willson, BT Ballantyne, and IM Davis. Hip strength in females with and without patellofemoral Phys Ther 1996; 76: 946–955. Onset timing of elec- patellar taping on stride characteristics and joint tromyographic activity in the vastus medialis oblique motion in subjects with patellofemoral pain.

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This patient is likely to have proliferative glomerulonephritis order ventolin 100mcg on line; the urine sediment of such patients exhibits significant proteinuria buy 100 mcg ventolin visa, RBCs buy cheap ventolin 100mcg line, and RBC casts buy 100 mcg ventolin. The differential diagnosis for proliferative glomerulonephritis includes connec- tive tissue diseases purchase ventolin 100mcg free shipping, systemic vasculitis, postinfectious glomerulonephritis, and other diseases. A 32-year-old woman presents to you after a recent hospital admission for flash pulmonary edema. She was diagnosed with hypertension several months ago. Her blood pressure remains poorly controlled despite compliance with a regimen of hydrochlorothiazide, amlodipine, and metoprolol. Her physical examination is remarkable for a blood pressure of 204/106 mm Hg in the left arm and bilateral abdominal bruits. You consider the diagnosis of renal artery steno- sis (RAS) secondary to fibromuscular dysplasia (FMD). Which of the following statements regarding RAS and FMD is true? Renal ultrasonography should be the first step in the evaluation of RAS because a finding of symmetrical kidneys precludes the need for further testing B. Angioplasty with stenting has become the most common method of managing FMD associated with hypertension and renal insufficien- cy; this procedure completely cures more then 50% of patients with hypertension and improves renal function in over one third C. The segmental nature of medial fibroplasia, the most common sub- type of FMD, results in the classic so-called beads-on-a-string appear- ance in the proximal third of the main renal artery D. Surgical repair of aneurysms is required if their diameter is greater than 1. It affects the distal two thirds of the main renal artery and its branches. In patients with a compatible clinical picture, evaluation for RAS starts with renal ultrasonography to measure kidney size. Even if the ultrasound scan shows that the kidneys are equal in size, further diagnostic testing is required. The choice of procedures is determined by the level of renal function: patients with a serum creati- nine level below 2 mg/dl should undergo renography; those with a serum creatinine above 2 mg/dl should undergo magnetic resonance angiography (MRA). The gold stan- dard for the diagnosis of RAS remains a renal arteriogram. Percutaneous intervention has been the standard of care, but large comparative trials are not feasible, given the relative rarity of these conditions. Angioplasty and stenting completely cure hypertension in about 22% of patients. Surgical repair of aneurysms (the “beads” seen on arteriography) is required if their diameter is greater than 1. A 58-year-old man known to have nephrotic syndrome presents to the emergency department. For sev- eral days, he has been experiencing low back pain and for the past several hours, he has been experi- encing hematuria and shortness of breath. The patient is tachypneic, with an oxygen saturation of 92% on 4 L of oxygen via nasal cannula. For this patient, which of the following statements regarding renal vein thrombosis (RVT) is true? RVT is most frequently associated with idiopathic and secondary membranous nephropathy; of these patients, 30% may have RVT 10 NEPHROLOGY 17 B. In addition to acute lower back pain and hematuria, most patients present with some degree of renal insufficiency C. Doppler ultrasonography is the most common modality used in the diagnosis of RVT D. For patients with RVT, a 6-month course of warfarin is indicated Key Concept/Objective: To understand the prevalence, clinical presentation, diagnostic modal- ities, and treatment of RVT RVT has been most frequently associated with idiopathic and secondary membranous nephropathy; 30% of these patients may have RVT. Pulmonary embolism may develop in up to 30% of patients with RVT, although alarmingly, the vast majority of these patients are asymptomatic.

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