Unless subsequent long-term complications such as pouchitis arise, patients generally undergo flexible fiberoptic pouchoscopy with surveillance biopsies of the ileal pouch approximately every 5 years.
The small bowel is typically affected the least and can maintain organized peristaltic contractions throughout the perioperative period. The stomach usually regains a normal pattern of emptying in 24 hours and the colon is last to regain motility usually in 48–72 hours.
Working group national high blood pressure education program (1996)
The following are the recommendations by this committee on BP measurement:
2. Cuff width-approximately 40% of arm circumference
3. Diastolic pressure-K5 for both child and adult
4. Number of measurements-average of 2 or more
Small cuff increases both systolic and diastolic BP.
Non compressible arteries (Monckeberg sclerosis) will produce pseudohypertension.
American heart association updates recommendations for blood pressure measuremens:-
· Systolic and diastolic blood pressures, as opposed to pulse pressure, remain the best means to classify hypertension. Cardiovascular risk begins to increase steadily as blood pressure rises from 115/75 mm Hg to higher values.
· 15% to 20% of patients with stage I hypertension have elevated blood pressure only in the presence of a physician. This "white-coat" hypertension is more common in older men and women, and antihypertensive treatment in these patients may reduce office blood pressure white not affecting ambulatory blood pressure.
· Patients who are older or have a long history of diabetes may have noncompressible brachial arteries , producing pseudohypertension.
· Orthostatic hypotension is defined by a decrease in systolic blood pressure of 20mm Hg or more or diastolic blood pressure of 10mm Hg or more after 3 minutes of quite standing after being supine. Food ingestion, time of day, age, and hydration can impact this form of hypotension, as can a history of Parkinsonism, diabetes, or multiple myeloma.
· Korotkoff sounds should be used to measure blood pressure. Compared with intra-arterial blood pressure, these sounds generally produce reduced systolic blood pressure values and higher diastolic values.
· Mercury sphygmomanometers are considered a gold standard for blood pressure measurement, but they are being supplanted by aneroid sphygmomanometers.
· Oscillometric blood pressure, in which the mean intra-arterial pressure is measured by the point of maximum of oscillation of the sphygmomanometer needle, affords the advantage of more accurate readings in noisy environments. However, this method is inappropriate for patients with noncompressible arteries.
· Posture:-Before performing a blood pressure reading, the patient should be comfortably seated with the back and arm supported, the legs uncrossed, and the upper arm at the level of the right atrium.
· Proper cuff size selection is critical to accurate measurements. The bladder length and width of the cuff should be 80% and 40%, respectively , of the arm circumference. Blood pressure measurement errors are generally worse in cuffs that are too small vs those that are too big.
· Blood pressure measurement in the sitting and recumbent positions is acceptable. The diastolic blood pressure can be expected to be about 5mm Hg higher in the sitting position.
· A different in blood pressure between the two arms can be expected in about 20% of patients. The higher value should be expected in about 20% of patients. The higher value should be the one used in treatment decisions.
· When measuring blood pressure, the cuff should be inflated to 30mm Hg above the point at which the radial disappears. The sphygmomanometer pressure should then be reduced at 2 to 3 mm/second. Two readings should be performed at least one minute apart.
· Blood pressure readings in the emergency department do not accurately predict hypertension on subsequent clinic visits.
· Blood pressure machines stationed in public places are frequently inaccurate.
· Five of 24 self-monitoring blood pressure systems have been shown to provide consistent, accurate results. Home blood pressure should be at least lower than 137/85 mm Hg. Night time home blood pressure is usually lower than daytime pressure.
Clinical Characteristics and Presentation
EpidemiologyThe average age of patients with idiopathic PAP in four recent case series comprising 140 patients was 42.6 years (range 6-73) with the majority being between 20 and 50 years old.[2,7-9] There was a male predominance with a male to female ratio of 2.7:1. These figures are similar to earlier reports.[2,10] Smokers outnumber nonsmokers by 3:1 in series that reported smoking status.[2,8] Although cases from Canada, Japan, and Israel were reported soon after the original description,[11-13] the two most recent case series from the United States (58 total patients)[2,7] contained only Caucasian patients. Whether this represents a true racial pattern of disease is unclear. Familial patterns have been reported primarily in congenital PAP  with one report in adult siblings with an immunoglobulin deficiency. Although the incidence and prevalence remain unknown, PAP is likely a rare disorder. A report from Israel suggests a prevalence of 3.7 per 106, whereas one from Pittsburgh estimates an ther incidence of 2 per 107 per year.
PAP has been associated with many conditions. Hematologic malignancies, predominantly myelogenous leukemias, are well established secondary causes.[18-21] Similarly, several exposures, including silica, aluminum, titanium, and nitrogen dioxide have been reported to precede the development of PAP.[22-26] Infections such as pneumocystis carinii pneumonia (PCP) (with or without acquired immunodeficiency syndrome),[27,28] mycobacteria,[29-32] nocardia, cytomegalovirus, and anaerobes have also been associated with PAP. These infections are occasionally felt to precede the development of PAP, but often are seen complicating the disease. This is supported by reports of decreased incidence of most infections with earlier recognition and treatment of PAP. Other reported associations include amyloidosis, and lung transplantation.
Clinical PresentationThe clinical presentation is variable and nonspecific. Up to 30% of patients may be asymptomatic. Others present with acute symptoms suggestive of pneumonia. Still others present with subacute or chronic symptoms and nonresolving infiltrates. The nonspecific presentation may lead to months or years of misdiagnosis.
Symptoms are frequently milder than expected from radiographic findings. Dyspnea and cough are the most common presenting symptoms. Chest pain, hemoptysis, fever, and weight loss are variably reported (Table 1).[1,2,7-9]
The physical exam is likewise nonspecific. Crackles, clubbing, and cyanosis all may be detected (Table 1).[1,2,7-9] Uncommon but reported features include pneumothorax and cor pulmonale.[39,40]
RadiologyMany patterns of chest x-ray (CXR) abnormalities have been described. Typically a bilateral, symmetric alveolar filling pattern is seen. Infiltrates classically are perihilar, extending to the periphery (lower > upper) sparing the costophrenic angles, yielding a "butterfly" distribution (Fig. 1). The pattern is similar to that of pulmonary edema and PCP. This typical picture seems somewhat less common in the latest case series.[2,7,41] Interstitial, mixed alveolar and interstitial, nodular, asymmetric, and focal patterns have all been described.[2,7-9] Although the absence of pleural effusions, adenopathy, and cardiomegaly help to narrow the differential diagnosis of the CXR pattern, the appearance is generally nonspecific.
CXR and high-resolution CT (HRCT) appearances have been shown to correlate with the presence of a restrictive ventilatory defect, reduced diffusing capacity, and hypoxemia. Although HRCT correlated somewhat better, the difference was not significant enough to justify the use of CT for follow-up.
Cases using gallium and Xenon-133 scintigraphy have been described but do not have practical applications in the routine diagnosis or management of PAP.
Physiological TestingIn general, the most common pulmonary function dis-order is restriction. The forced vital capacity (FVC) and total lung capacity (TLC) are mildly reduced in most series with a disproportionate reduction in diffusing capacity. Patients tend to be mildly hypoxemic with an elevated alveolar-arterial oxygen gradient and a compensated respiratory alkalosis (Table 2).[2,7-9] The shunt fraction has been shown to be elevated as compared to patients with other diffuse lung diseases.
The severity of pulmonary function abnormalities has varied in different case series. This may in part be explained by differences in the proportion of patients requiring whole lung lavage and mortality in these case series, suggesting the populations differed.
Diagnostic EvaluationAlthough history, physical examination, radiographic studies, and physiological testing may suggest PAP, further evaluation is usually needed to confirm the diagnosis. Blood tests, sputum, and BAL results similarly support the diagnosis, however transbronchial or open lung biopsy remain the standard. Table 3 summarizes the clinical and diagnostic features of PAP.
Serum lactate dehydrogenase (LDH) levels have been shown to be elevated relative to other diffuse lung diseases. These levels are also increased in BAL fluids and have some correlation with the A-a gradient. Krebs von den Lungen-6 (KL-6), a mucin-like protein secreted from type II pneumocytes, has been reported to be elevated in serum and BAL fluids of PAP patients. The levels were higher than for interstitial lung diseases and correlated with disease activity, radiographic appearance, and pO2.
49,50 Surfactant protein A (SP-A) is elevated in sera of PAP and idiopathic pulmonary fibrosis patients compared with control and other lung diseases. It is similarly elevated in the sputum, but not all patients can expectorate. Surfactant protein D (SP-D) is also elevated in the sera of PAP patients and has been shown to correlate with disease severity. It was similarly elevated in idiopathic pulmonary fibrosis and interstitial lung disease associated with collagen vascular diseases.
BAL findings of PAP have occasionally been used as diagnostic.[7,8] Typically, however, tissue is obtained. Traditionally tissue was obtained by open lung biopsy. It has been suggested that transbronchial biopsy can be used to accurately make the diagnosis. Recent case series support this, with a trend toward a greater percentage of cases being diagnosed with transbronchial biopsy.[2,8]
HistopathologyAnalysis of BAL fluid has revealed increased concentrations of lipids and proteins, particularly those associated with surfactant. The classes and distribution of surfactant phospholipids do not substantially differ from normals,54,55 though there is much variability. Cholesterol concentrations are consistently elevated.[54,55] Surfactant protein levels vary considerably from patient to patient though they are consistently elevated. SP-A has been reported to increase out of proportion to the total protein though this is variable. The SP-A: phospholipid ratio is also elevated.[55,57] SP-B is similarly increased, with an increased SP-B:phospholipid ratio and a normal SP-B:SP-A ratio. A dimeric form of surfactant associated protein C is present in increased quantities. SP-D has also been identified to be elevated in PAP patients though to a smaller degree than other surfactant associated proteins. This accumulated PAP material has been shown to have normal surface activity after processing.
A small study of cells in the BAL fluid revealed an increase in both the CD4 and CD8 lymphocyte population. The CD4:CD8 ratio tended to be high but was variable.
Macroscopically, both biopsy and necropsy specimens reveal multiple, up to 2-3 cm, yellow-gray nodular areas of consolidation. These areas are firm and exude a fatty substance when cut.[1,7,11] Microscopically, the alveoli are filled with a granular and floccular acidophilic material that is periodic acid Schiff (PAS) positive (Fig. 3). This substance is seen to extend to the small bronchioles at times. Staining these specimens for surfactant specific apoprotein reveals uniform uptake in primary PAP and patchy uptake in secondary PAP. Alveolar and interstitial architecture are typically preserved, though infiltration of bronchial walls with lymphocytes and thickening or fibrosis may be seen. The vasculature appears normal.[1,7,11] Electron microscopy reveals characteristic multilamellated structures within the alveolar material as well as inclusions in the cytoplasm of macrophages.[7,62] Papanicolaou-stained specimens from PAP contain scattered, amorphous, or granular globules that are PAS positive and contain these multilamellated structures when examined ultrastructurally