Peds Rot Endocrine
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| the most common form of short-limbed dwarfism (also autosomonal dominant; trunk is of nl length, results from amutation in the gene that codes for fibroblast growth factor receptor 3 (FGFR3). ) | Achondroplasia (classic condrodystrophy). Upper arms and thighs are proportionately shorter than the forearms and legs. intelligence and sexual fxn are nl. Tx: GH, limb lengthening is controversial, but possible
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| An autosomal recessive disorder of mucopolysaccharide storage with skeletal abnormalities that include: shortening of the spine, kyphosis, scoliosis, shortened extremeties, pectus carinatum,genu valgum, & hypoplastic odontoid with atlantoaxial instability | Ostochondrodystrophy (Morquio Dz). Tx: Prevention of cervical myelopathy. Bone marrow transplantation has been successful in alleviating some of the sx
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| The most common type of idiopathic scoliosis curve is | right thoracic scoliosis.
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| Idiopathic scoliosis generally begins at which age? | 8-10 years and usually progresses during growth. 4-5x more likely in girls than boys.
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| Scoliosis curvatures of less than ___ degrees do not require tx unless they show progression | 20. Curvature of 20-40 degrees require bracing. >40 does not respond to bracing and may require surgery
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| Describe the pathophysiology of Diabetes Type I. | Lack of insulin production from the beta cells of the pancreas. Loss of major anabolic hormone, insulin, causes catabolic state to ensue. Decreased peripheral glucose utilization and increased hepatic glucose production by gluconeogenesis & glycogenolysis
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| Pathophysiology of Diabetes Type 1 continued | Lack of insulin prevents glucose from entering cell and hyperglycemia results. Catabolic mediators (glucagon, epinephrine, GH, and cortisol) bring about production of ketoacids by triggering lipolysis, FA release and ketoacid synthesis.
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| Pathophysiology of Diabetes Type 1 continued | When blood glucose concentration exceeds 180 mg/dL, the resultant glycosuria causes an osmostic diuresis with polyuria. If insulin def is severe, ketones are produced, the blood's native buffering system is overwhelmed, and DKA ensues
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| Characteristics of Diabetic Ketoacidosis | hyperglycemia, metabolic acidosis (ketoacidosis), dehydration, and lethargy. Polyuria, polydipsia, fatige, HA, N/V, abdominal pain, tachycadia, hyperpnea, fruity breath
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| Fatal complication of DKA | cerebral edema. Sx: changing mental status, unequal pupils, decorticate or decerebrate posturing, and/or seizures
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| Treatment for DKA | Fluid resuscitation: nl saline or lactated Ringer solution, 10mL per kg IV colus. Fluid deficit replaced over 48 hrs. Insulin drip .1U/kg/hr. Goal is to dec serum gluc 50-100mg/dL/hour
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| Complications from diabetes | microvascular disease of the eye (retinopathy), kidney (nephropathy), and nerves (neuropathy). Microvascular dz not seen until child has been insulin dependent for at least 10 years. Athersclerosis leading to MI or stroke
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| Annual screening for diabetic kids | annual urine collections to screen for microalbuminuria, annual opthalmologic exams, and annual screening for hyperlipidemia
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| Standard insulin tx initiated in Type I DM | .5-1.0 U insulin per kg/day. 2/3 of daily dose before breakfast, 1/3 before dinner. Human insulin is divided between short acting Humalog insulin and intermediate acting neutral protamine Hagedorn (NPH) insulin.
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| New onset of weight loss, polydypsia, polyphagia, and polyuria is consistent with type __ DM | I
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| The most common type of diabetes in people younger than 40 years | Type I DM (immune related). Type II is not immune related
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| ___ grams of glucose usually covers 30 minutes of exercise in the Type I DM | 15grams
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| When to dose adjust the Type I DM child | If more than 50% of the values are above the desired range for age, or more than 14% below the desired range. Children with DM should have clinic visits every 3 months
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| Blood sugars should be measured how many times a day in diabetics? | 3-4 times daily
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| Sx of hypoglycemia | hunger, weakness, shakiness, sweating, drowsiness, HA, and behavioral changes.
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| Tx of hypoglycemia | 4oz of juice, sugar containing soda or milk, and waiting 10 minutes. If blood gluc still below 60mg/dL, reapeat. If above 60mg/dL, solids are given
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| Tx of DM II | If HbA1C is still near nl and ketones are not increased, then lifestyle changes are firstline. If HbA1C is 6.2-9%, then oral hypoglycemia agent (metformin 250-500mg/day in children>10yo). If presentation is more severe, then IV or SQ insulin.
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| Some of the lab studies evaluated with an overweight BMI child | fasting lipid profile, insulin and glucose, liver function tests, thyroid function.
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| For children 2-7 with a BMI at 95% the goal should be | maintenance of baseline weight, allowing the child to "grow into" his or her height, with a gradual normalization of BMI.
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| For children >7 with BMI between 85-95%, without complications, the goal is | weight maintenance. If secondary complications, then the recommnended weight loss is 1lb/month until a BMI of less than 85% is achieved
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| ____ is a slective serotonin reuptake inhibitor and is approved for patients ___ and older | sibutramine; 16 yo
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| ___ is a lipase inhibitor and is approved for paitnets over ___ years | Orlistat; 12 yo
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