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Originally described in 1967 by Northway, a radiologist, radiographic findings remain a major feature of the diagnosis and staging of the disease process. The older definition describes infants who remain oxygen dependent at 28 days of age or more (typically following a course of mechanical ventilation) and with abnormal radiographic findings. With the understanding that it is not "normal" for a 26 week gestation fetus to be outside of a fluid filled intrauterine environment, it is easy to appreciate how endotracheal intubation and mechanical ventilation can "do harm". All aspects of patient care, nutrition, fluid administration, respiratory, infectious disease, etc. If continued unchecked, the final outcome is a chronic pulmonary condition with features that include increased airway resistance and reactivity, mucous production, cystic emphysema, scarring, and atelectasis. In the early stages, tachypnea, retractions, cyanosis, and occasional grunting are seen. Prevention strategies are by far the most effective and satisfying in decreasing the incidence and reducing the severity of chronic lung disease. Other approaches to prevention are listed in Table 2 (2), the most recent of which has been vitamin A supplementation. Vitamin A, necessary for epithelial integrity and healing, is often Page - 307 deficient in preterm infants, (as are other cofactors, antioxidants, minerals, and trace elements). Recent studies have shown that vitamin A supplementation is beneficial in ameliorating the development and progression of chronic lung disease (3). Because lung growth and development continues throughout infancy and early childhood, this is a disease process that the patient literally (completely or partially) outgrows. The goals of effective supportive therapy are to achieve adequate nutrition and growth while limiting episodes of disease exacerbation. It is, therefore, easy to understand why nutritional support serves as the mainstay of treatment. Parents share in the frustration of providing these infants with enough calories to grow and often suffer feelings of guilt in the process. Special formulas have been developed to increase caloric density (and intake), provide a proper balance of carbohydrate, protein, and fat, and limit free water. Indwelling nasogastric feeding tubes, and in some cases, gastrostomy tubes, are placed to provide enteral calories in infants with varying degrees of feeding difficulties. The rationale for diuretic use early in the disease process is to treat the pulmonary edema that accompanies inflammation and capillary leak. Furosemide is the first line and most popular diuretic due to its additional benefits of venodilation and diminished airway reactivity. However, because of the multiple untoward side effects of furosemide, chlorothiazide (with or without spironolactone) is frequently used in "maintenance" therapy. Rarely, in the most severe cases, theophylline may be employed as an adjunct to inhaled agents. It is often beneficial to auscultate the chest before and several minutes following an inhalation treatment to determine its clinical efficacy. Confirming therapeutic benefit in the individual patient is important for determining ongoing management. Airway disease in these infants may sometimes be unresponsive to bronchodilator therapy. The anti-inflammatory and pro-surfactant properties of corticosteroids made them a logical focus of study. These and subsequent studies have repeatedly demonstrated the positive short term benefits of corticosteroids as manifested by dramatic weaning of ventilator and oxygen support. As with many clinical trials, dosing amount, frequency, and treatment duration varied widely among studies. Adverse side effects, including hyperglycemia and hypertension, have also been documented. Subsequent trials of early dexamethasone use (within the 1st week of life) have shown greater risk than benefit (6,7). Therefore, if dexamethasone therapy is being considered, its use should be reserved for those patients with established chronic lung disease or prolonged ventilator dependency, typically older than 1 week of age (8,9). Of great concern is evidence suggesting that dexamethasone treatment is associated with an increase in developmental disability and cerebral palsy.

Diseases

  • Choroiditis, serpiginous
  • Finnish type amyloidosis
  • Hypoparathyroidism short stature
  • Factor V Leiden mutation
  • McDowall syndrome
  • Iritis
  • Cat scratch disease
  • Tibia absent polydactyly arachnoid cyst
  • Diabetes insipidus, nephrogenic type 2

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The depth of transmural involvement and presence of extrarectal extension can be accurately assessed. The sound waves are bounced back to the transducer and are electronically converted into a pictorial image. Instruct the patient that a small-volume rectal enema may be required approximately 1 hour before the ultrasound examination. Significant barriers, such as prostate glandular tissue and vascular structure, are interposed between the prostatic lumen and the bloodstream. Levels greater than 4 ng/mL have been found in more than 80% of men with prostate cancer. A positive screening test often triggers a biopsy and even potential life-threatening surgery with very little benefit. However, high-risk men such as those of African American descent or with a genetic predisposition. Prostatic-specific membrane antigen may, with further study, represent an excellent marker for prostate cancer. P 758 prostate-specific antigen these biomarkers can be detected in the urine of prostate cancer patients after a short period of professional prostate massage. Furthermore these biomarkers are not influenced by patient age or prostate volume. They are the most significant component contributing to the osmotic pressure in the vascular space. This osmotic pressure keeps fluid in the vascular space, minimizing extravasation of fluid. Albumin and globulin constitute most of the protein in the body and are measured together as the total protein. The major effect of albumin in the blood is to maintain colloidal osmotic pressure. Furthermore, albumin transports important blood constituents, such as drugs, hormones, and enzymes. Albumin is synthesized in the liver and is therefore a measure of hepatic function. When disease affects the liver cell, the hepatocyte loses its ability to synthesize albumin. Some transporting proteins, such as thyroid and cortisol-binding globulin, also contribute to this electrophoretic zone. Alpha2 globulins include serum haptoglobins (which bind hemoglobin during hemolysis), ceruloplasmin (which is a carrier for copper), prothrombin, and cholinesterase (which is an enzyme used in the catabolism of acetylcholine). Beta1 globulins include lipoproteins, transferrin, plasminogen, and complement proteins; beta2 globulins include fibrinogen. Malnourished patients, especially after surgery, have a greatly decreased level of serum proteins. Burn patients and patients who have protein-losing enteropathies and uropathies have low levels of protein despite normal synthesis. In some diseases, albumin is selectively diminished, and globulins are normal or increased to maintain a normal total protein level. Albumin, a molecule that is generally smaller than globulin, is selectively lost into the extravascular space. Another group of diseases similarly associated with low albumin, high globulin, and normal total protein levels is chronic liver diseases. In these diseases, the liver cannot produce albumin, but globulin is adequately made in the reticuloendothelial system. In both of these types of diseases, the albumin level is low but the total protein level is normal because of increased globulin levels. These changes, however, can be detected if one measures the albumin/globulin ratio.

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These children should also be given a self-administered epinephrine kit with instructions and a demonstration of its use. Scorpions from other parts of the world do contain venoms, which can be substantially toxic. Avoidance of stinging arthropods becomes an important part of management and includes: identification and elimination of stinging insect nests, avoiding brightly colored clothing or strongly scented lotions, wearing shoes or protective footwear outdoors, exercising caution around sites frequented by stinging insects (eaves, attics, and areas where food is present outside), and wearing protective clothing when outside (long shirt, pants, hat, gloves, socks and shoes) (6). Marine envenomations common in Hawaii occur from box jellyfish, Portuguese man-of-war, and venomous fish. These animals produce protein-based venoms that are used in self-defense or to capture prey. Unfortunately the unwary beach goers may interact adversely with these animals and sustain intensely painful wounds. As a general rule, these venoms tend to be heat labile and can be denatured with heat. A twelve year old male moving boxes in the basement experienced a pinprick sensation on his right hand followed by muscle cramps and swelling in his right axilla. True/False: Repeat anaphylactic reactions to insect stings are more common in adults than in children. True, anaphylaxis can occur from any repeated insect bite or sting in which re-exposure to an antigen occurs. Common Skin Conditions Annemarie Uliasz this is a 6 month old female who is brought to the office with her mother with a chief complaint of a diaper rash for one week. Mother has been using baby powder to keep the area dry, but the rash is worsening. Upon examination, the buttocks, perianal region, and tops of the thighs appear erythematous with no ulcerations or erosions. Areas of flexure are involved and there are some beefy red areas with a few satellite lesions. Baby powder does not keep the area dry once the child urinates, so its value is minimal. Petrolatum or zinc oxide applied to the diaper region is suggested as prophylaxis against irritation. Topical clotrimazole cream is also recommended to eliminate any yeast infection that may be present. The most superficial layer of the epidermis, the stratum corneum, serves as a protective barrier against the environment, and prevents desiccation. Damage to the epidermis increases skin permeability, thereby increasing the risk of infection. The dermis consists of collagen, elastin, and proteoglycans, which lend support and durability to the skin. Blood vessels, lymphatics, sweat glands, hair follicles, smooth muscle, and neuroreceptors are all found in the dermis. Fibroblasts in the dermis are responsible for collagen production and are the predominant cell in this layer of the skin. Blood vessels and lymphatics are found in the subcutaneous tissue as well as the base of hair follicles and sweat glands. In order to describe a skin lesion, one must have a basic understanding of the language of dermatology. A primary lesion is a lesion that has not been altered by trauma, infection, scratching, therapy, or regression over time. Primary lesions are described as macules, patches, papules, nodules, tumors, vesicles, bullae, pustules, plaques, cysts, and wheals. A macule is a flat, circumscribed skin discoloration that is neither raised nor depressed. Tumors are usually larger in diameter than nodules, and tend to be variable in consistency and mobility.

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The duration of distress with mild to moderate aspiration syndromes is from several hours to days. Aspiration can occur in utero or during the intrapartum period as well as during the early postpartum period. Since meconium aspiration is the most common problem, much effort has been made over the last 30 years to prevent this disease by reducing intrapartum and postpartum aspiration. Thorough suctioning of the oropharynx with a large bore catheter upon the delivery of the head is typically performed by the obstetrician. The pediatrician, needs to assess the quality of the meconium (thin, moderate or thick) and the state of the newborn before determining what is needed after birth. A large randomized trial has confirmed that aggressive intubation is not necessary for most infants with meconium in the amniotic fluid. The sudden onset of significant respiratory distress should raise the possibility of an air leak syndrome. The most common air leak syndromes are pneumomediastinum, pneumothorax and pneumopericardium. In addition to respiratory distress, a severe air leak condition may cause hypotension (due to decreases in cardiac output), muffled heart tones, abdominal distention, asymmetric chest shape and deviation of the cardiac sounds. Chest radiographs are diagnostic with free air in the hemithorax and a visible edge of the collapsed lung. The elevation of the thymus with a sail or bat wing sign suggests a pneumomediastinum. Hypotension and bradycardia occur rapidly in a tension pneumothorax or pneumopericardium (cardiac dysfunction is due to reduced venous return due to compression of the heart and mediastinal vascular structures). Treatment of significant air leak syndromes requires immediate air evacuation (thoracentesis or pericardiocentesis) with a needle or small catheter, followed by chest or pericardial tube insertion. In cases other than a bronchopleural fistula, the air leak will usually seal within a few days. Most infants are less than 34 weeks gestation and the incidence and severity increase with decreasing gestation age. These premature infants have progressively more severe respiratory distress after birth. The classic findings of cyanosis, grunting, nasal flaring, intercostal and subcostal retractions and tachypnea are present. The chest radiograph reveals decreased lung inflation with diffuse symmetrical reticulogranular (ground glass appearance) lung fields and air bronchograms. The presence of apnea suggests severe disease accompanied by refractory hypoxemia and acidosis. Without surfactant, the surface tension of the alveolar sacs is high, leading to an increased tendency of the alveoli to collapse. This relationship states that as the radius of the air filled alveolus decreases, the pressure within the alveolus increases. This increased pressure requires an equivalent external opposing pressure to keep the alveolus inflated. Without the opposing pressure, the gas under this pressure is forced out of the alveolus. If the alveolus is connected to an adjacent alveolus with a larger radius, air will preferentially inflate the larger alveolus and ultimately collapse the smaller alveolus. This leads to the network of air-filled alveoli juxtaposed to atelectatic alveoli and creates the reticulogranular pattern (ground glass appearance) of the lung. The air bronchograms are created by atelectatic alveoli outlining the adjacent rigidly distended airways. The phospholipids and surfactant related proteins, contained in surfactant, spread along the air liquid interface to decrease alveolar surface tension. Furthermore, the surfactant molecules contribute to the larger alveoli developing a higher surface tension during inspiration and a lower surface tension (as the alveoli deflate) during expiration when the surfactant molecules become more compact along the air liquid interface. Today, several types of animal based surfactants have been approved for clinical use.