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Menno Terriet, M.D.

• Department of Anesthesia
• Veterans Affairs Medical Center
• Miami, FL

Is the uid serous blood pressure normal low cheap aldactone, mucinous blood pressure chart table generic aldactone 100 mg without prescription, or hemand designate their location as either cortical blood pressure pulse rate order discount aldactone, orrhagic If both blood pressure chart low diastolic buy discount aldactone 25mg on line, document the percentage of each pearance of the ovary will vary considerably region. Examine the surfaces of the cysts for eviwith the age and the reproductive status of the dence of granularity, nodules, or papillary projecwoman. The thickness of the cyst walls should also years can measure up to 4 cm, whereas an ovary be recorded. Describe any regions of hemorrhage this size in a postmenopausal woman warrants or necrosis. If fallopian tube were removed as a prophylactic a stromal or steroid cell tumor is suspected, tisprocedure in a woman with a family history of sue should be saved frozen in case fat stains are ovarian or breast carcinoma, the entire ovary and needed. The most common can aid in documentation of the mass and for indication is for the removal of a dermoid cyst. At this After weighing and measuring the cyst, examine point, it may be helpful to x the 1-cm slices in the external surface for evidence of rupture. Place the cyst in a container, and carefully make Historically, ovarian tumors are submitted a small incision in the wall to allow its contents with a minimum of one section per 1 to 2 cm of to be drained. Continue the incision with a pair cially useful in the case of mucinous tumors, of scissors to expose the entire inner surface. The which tend to have only focal regions demonstratthick sebaceous uid within a dermoid cyst may ing atypical or frankly invasive elements. If the have to be removed by rinsing briey with hot tumor is uniform throughout, as many serous water. Examine the cyst lining, and look for any tumors are, fewer sections may be prudent. Cysts that show granular, nodular, or papillary this region and any other thickened areas should excrescences should be thoroughly sampled. Ovary and Fallopian Tube 165 include any regions that appear sieve-like or Lymph nodes are received separately and deshoneycombed. Sections that routine manner for evaluation of metastatic disdemonstrate the junction between the ovary and ease, as detailed in chapter 5. Omentectomy specimens should be weighed, Metastatic involvement is suggested by the measured, and serially sectioned at 0. Five representative sections are usually suftralateral ovary and/or the serosa or parencient, although some authorities recommend up chyma of the uterus Is the tumor extensively involves the pelvic peritotumor microscopic, 2 cm or less, or more than neum, a cavitronic ultrasonic surgical aspirator 2 cm P rod cts of on cep tion an d lacen tas 2 Products of Conception are condent of your identication of villi, submit representative sections in two or three tissue cassettes. However, because the conrmation of an Products of conception is the term used for intraintrauterine pregnancy is often needed immediuterine tissue that is either passed spontaneously ately, it may be wise to use the following guideor removed surgically in early gestation. These line: Submit the entire specimen if it is small or specimens are usually sent for diagnostic or theras much as can be included in ve tissue cassettes. The major goal is to verify that a Always specify the percentage of the specimen gestation was present. This requires the identithat was submitted, and include only tissue cation of either fetal parts, chorionic villi, or trofragments. The presence of decidua alone is evaluation of blood clots from intrauterine pregnot sufcent for diagnosis. If and placental neoplasia may also be identied, no villi are identied after your initial microalthough these are much less common. In the case of a clinically suspected molar Specimens from rst trimester gestations are pregnancy or the presence of hydropic villi, the usually composed of irregularly shaped small submission of at least eight tissue cassettes is tissue fragments and blood clots suspended withrecommended to assess the degree of trophoblast in a uid-lled container. Any large tissue fragments, that is, tents of the container, and give an estimate of the fragments greater than 3 to 4 cm, should be secamount of the specimen by volume (in cubic tioned and entirely submitted if they are rm, centimeters) or as an aggregate measurement. Consider sending fresh Spread the specimen across your work bench, tissue for ow cytometric ploidy analysis or and separate the blood clots from the tissue. Partial moles fully inspect the tissue for fetal parts and vilare triploid, whereas complete moles are diploid lous tissue. Uterine resection specimens for whereas decidua is more likely to be rmer and gestational trophoblastic malignancies should membranous. Another method of examination is be handled as for hysterectomies for endometrial to suspend the tissue fragments in saline. The or cervical cancer depending on the site of the delicate villous fronds will then become readily tumor. Also, look for evidence of swollen or Second trimester therapeutic or elective aborhydropic villi, which appear as small, grapetion specimens may have intact placentas and like vesicles. If no fetal parts are identied and you autopsy is beyond the scope of this chapter; 166 29. Products of Conception and Placentas 167 however, most cases can be appropriately hancircummarginate (a smooth chorionic surface dled with a limited approach. Examine the external appearance may reect previous bleeding from earlier plafor skin slippage and any gross abnormalities of cental separation. Begintion of the internal organs, and take a piece of ning at the ruptured end, roll the membrane strip liver, lung, and gonads for microscopic evaluawith the amnion inward around a small probe. The membranes can now be removed by the placenta can be routinely handled, as detrimming them along the placental margin. Although the length provided may be articially shortened if a segment was removed in the delivery room, excesPlacentas sively short (less than 30 cm) or long (more than 70 cm) cords are signicant because of their assoPlacentas are submitted for evaluation because ciation with abnormal fetal development and of maternal conditions, fetal/neonatal condiactivity. Insertions at the edge of the placenta tions, or gross anomalies of the placenta and in or in the membranes may be associated with all multiple gestations. Many abnormalities can exposed vessels, which should be examined be recognized with a thorough gross examinacarefully for any tears or thrombi. Approach each placenta by systematically umbilical cord at its insertion, and examine the evaluating the three main components: the fetal entire length of the cord for thinning, thrombi, membranes, the umbilical cord, and the placenor knots. There should be allows for the drainage of blood and uid, which two small thick-walled arteriesand one large thinis copiously expressed from the placental bed on walled vein. Always be aware of the clinical hisjoin together and they may not be fused into their tory before proceeding, and check the contents of terminal vessel until just above this point. Also, the container in which the placenta was received twisted regions of the umbilical cord can give the for any separate blood clots. Orient the placenta articial appearance of an increased number of by placing the spongy, red maternal surface face vessels on cross section. Therefore, for an accurate down and the shiny, membranous fetal surface documentation of the number of vessels, it is best with umbilical cord face up. Invert the memto submit a transverse section of the umbilical branes, if necessary, so that they are draped cord for examination from an area that is not around the fetal surface. Normal membranes should be shiny and clear Record its weight and three-dimensional meaand should insert at the edge of the placental surement. Examine the membranes on the inammation; small white nodules, which indifetal surface rst, and look for nodules within cate amnion nodosum; and meconium staining, or just below the amnion/chorion layer. As cial white nodules or ne granularity may repreillustrated, membrane insertion within the cirsent amnion nodosum, whereas rm, yellowish cumference of the fetal surface is called placenta nodules beneath the membranes may represent extrachorialis and can be subdivided into either subchorionic brin deposition. If present, these 168 169 170 Surgical Pathology Dissection should be sampled for histology. Next, examcan be readily recognized by the fact that they ine the vessels that radiate toward the umbilical lie on top of the veins. Turn the plamay be reected by one side being severely concenta over, and examine the maternal surface. Adherent clots without underlying compression do not necessarily signify a placental abruption.

Continuity of the duodenum with the stomach should be demonstrated to differentiate a distended duodenum from other cystic masses blood pressure medication cost buy aldactone on line amex, including choledochal or hepatic cysts blood pressure chart infants discount aldactone 100mg online. Prognosis Survival after surgery in cases with isolated duodenal atresia is more than 95% blood pressure garlic purchase 100 mg aldactone. Intrinsic lesions result from absent (atresia) or partial (stenosis) recanalization of the intestine hypertension kidney 100 mg aldactone fast delivery. In cases of atresia, the two segments of the gut may be either completely separated or connected by a fibrous cord. In cases of stenosis, the lumen of the gut is narrowed or the two intestinal segments are separated by a septum with a central diaphragm. Apple-peel atresia is characterized by absence of a vast segment of the small bowel, which can include distal duodenum, the entire jejunum and proximal ileus. The most frequent site of small bowel obstruction is distal ileus (35%), followed by proximal jejunum (30%), distal jejunum (20%), proximal ileus (15%). Anorectal atresia results from abnormal division of the cloaca during the 9th week of development. Prevalence Intestinal obstruction is found in about 1 per 2000 births; in about half of the cases, there is small bowel obstruction and in the other half anorectal atresia. Etiology Although the condition is usually sporadic, in multiple intestinal atresia, familial cases have been described. In contrast with anorectal atresia, associated defects such as genitourinary, vertebral, cardiovascular and gastrointestinal anomalies are found in about 80% of cases. Diagnosis the lumens of the small bowel and colon do not normally exceed 7 mm and 20 mm, respectively. Diagnosis of obstruction is usually made quite late in pregnancy (after 25 weeks), as dilatation of the intestinal lumen is slow and progressive. Jejunal and ileal obstructions are imaged as multiple fluid-filled loops of bowel in the abdomen. If bowel perforation occurs, transient ascites, meconium peritonitis and meconium pseudocysts may ensue. Polyhydramnios (usually after 25 weeks) is common, especially with proximal obstructions. When considering a diagnosis of small bowel obstruction, care should be taken to exclude renal tract abnormalities and other intra-abdominal cysts such as mesenteric, ovarian or duplication cysts. In anorectal atresia, prenatal diagnosis is usually difficult because the proximal bowel may not demonstrate significant dilatation and the amniotic fluid volume is usually normal; occasionally calcified intraluminal meconium in the fetal pelvis may be seen. Prognosis Infants with bowel obstruction typically present in the early neonatal period with symptoms of vomiting and abdominal distention. The prognosis is related to the gestational age at delivery, the presence of associated abnormalities and site of obstruction. In those born after 32 weeks with isolated obstruction requiring resection of only a short segment of bowel, survival is more than 95%. Loss of large segments of bowel can lead to short gut syndrome, which is a lethal condition. It derives from failure of migration of neuroblasts from the neural crest to the bowel segments, which generally occurs between the 6th and 12th weeks of gestation. Another theory suggests that the disease is caused by degeneration of normally migrated neuroblasts during either preor postnatal life. Etiology It is considered to be a sporadic disease, although in about 5% of cases there is a familial inheritance. Diagnosis the aganglionic segment is unable to transmit a peristaltic wave, and therefore meconium accumulates and causes dilatation of the lumen of the bowel. The ultrasound appearance is similar to that of anorectal atresia, when the affected segment is colon or rectum. Polyhydramnios and dilatation of the loops are present in the case of small bowel involvement; on this occasion, it is not different from other types of obstruction. Prognosis Postnatal surgery is aimed at removing the affected segment and this may be a two-stage procedure with temporary colostomy. Bowel perforation usually occurs proximal to some form of obstruction, although this cannot always be demonstrated. Etiology Intestinal stenosis or atresia and meconium ileus account for 65% of the cases. Meconium ileus is the impaction of abnormally thick and sticky meconium in the distal ileum, and, in the majority of cases, this is due to cystic fibrosis. Diagnosis In the typical case, meconium peritonitis is featured by the association of intra-abdominal echogenic area, dilated bowel loops and ascites. The diagnosis should be considered if the fetal bowel is observed to be dilated or whenever an area of fetal intraabdominal hyperechogenicity is detected. The differential diagnosis of hyperechogenic bowel includes: intra-amniotic hemorrhage; early ascites; fetal hypoxia; meconium peritonitis; and cystic fibrosis. The prevalence of cystic fibrosis in fetuses with prenatal diagnosis of intestinal obstruction may be about 10%. Prognosis Meconium peritonitis is associated with a more than 50% mortality in the neonatal period. Hepatic enlargement may also be caused by hemangioma, which is usually hypoechogenic, or hepatoblastoma (the most frequent malignant tumor in fetal life), in which there are areas of calcification. Prevalence Hepatic calcifications are found at mid-trimester ultrasonography in about 1 per 2000 fetuses. Etiology the vast majority of cases are idiopathic but, in a few cases, hepatic calcifications have been found in association with congenital infections and chromosomal abnormalities. Prognosis this depends on the presence of associated infection or chromosomal defects. Renal tract anomalies or dilated bowel are the most common explanations, although cystic structures may arise from the biliary tree, ovaries, mesentery or uterus. The correct diagnosis of these abnormalities may not be possible by ultrasound examination, but the most likely diagnosis is usually suggested by the position of the cyst, its relationship with other structures and the normality of other organs. Choledochal cysts Choledochal cysts represent cystic dilatation of the common biliary duct. Prenatally, the diagnosis may be made ultrasonographically by the demonstration of a cyst in the upper right side of the fetal abdomen. The differential diagnosis includes enteric duplication cyst, liver cysts, situs inversus or duodenal atresia. The absence of polyhydramnios or peristalsis may help to differentiate the condition from bowel disorders. Postnatally, early diagnosis and removal of the cyst may avoid the development of biliary cirrhosis, portal hypertension, calculi formation or adenocarcinoma. Ovarian cysts Ovarian cysts are common and they may be found in up to one-third of newborns at autopsy, although they are usually small and asymptomatic. Fetal ovarian cysts are hormone-sensitive (human chorionic gonadotropin from the placenta) and tend to occur after 25 weeks of gestation; they are more common in diabetic or rhesus isoimmunized mothers as a result of placental hyperplasia. The majority of cysts are benign and resolve spontaneously in the neonatal period.

Chemoembolization involves the use of ethiodized oil as a carrier for various cytotoxic drugs pulse pressure 70-80 order 25 mg aldactone overnight delivery. The encapsulation of drugs in microcapsules capable of slow deterioration is also of interest blood pressure chart when to go to the hospital purchase aldactone uk. In addition to vascular occlusion blood pressure chart 19 year old purchase genuine aldactone on line, encapsulation allows the slow release of cytotoxic agents in direct proximity to Management of neuroendocrine tumours 261 tumour deposits blood pressure 60 over 30 purchase 25 mg aldactone amex. A number of authors have reported their experience of these techniques, although it is uncertain whether there is any advantage over embolization alone. However, the development of modern cryotherapy delivery systems, together with the introduction of intraoperative ultrasound, has allowed the application of cryotherapy techniques for the treatment of hepatic tumours. Hepatic cryotherapy involves the delivery of liquid nitrogen to the tip of relatively thin insulated probes. Intraoperative ultrasound guides probe placement and the monitoring of ice formation during the freezing process. Cryotherapy has been widely used for the treatment of primary90 and secondary hepatic tumours, predominately colorectal metastases. Four patients were symptomatic and three of these patients had elevated tumour markers. Patients with elevated preoperative markers showed a dramatic reduction in tumour markers following treatment. This group published their experience of a total of 13 patients with neuroendocrine hepatic metastases treated by hepatic cryotherapy. One patient died of bronchopneumonia 45 months following cryotherapy, but without evidence of tumour recurrence. One patient developed a recurrence in one of seven liver metastases and this was subsequently treated by hepatic resection 13 months following hepatic cryotherapy. This patient went on to develop a sacral recurrence of his rectal carcinoid which was also resected. However, the remaining nine patients were alive with no evidence of recurrent disease. Seven of these 13 patients had had symptoms related to ectopic hormone production. In all patients symptoms were significantly alleviated and postoperatively five patients were completely asymptomatic. In this series, two patients with carcinoid metastases developed a coagulopathy postoperatively and required further laparotomy together with the replacement of clotting factors. All patients had advanced disease and cryosurgery was considered palliative as evidenced by residual liver disease, lymph node involvement, residual primary disease or unknown primary site. Recurrent symptoms following cryotherapy were effectively palliated in three patients using somatostatin and in five patients by chemotherapy. Cryotherapy has the advantage of being able to treat bilobar disease and lesions close to major blood vessels. This treatment appears to be safe and to provide good palliation of symptoms related to ectopic hormone production. In some patients it appears that longSurgical Management of hepatobiliary and pancreatic disorders 262 term disease-free survival may be obtained. However, given the long natural history of neuroendocrine tumours and the relatively short follow-up of patients treated by hepatic cryotherapy, evidence for prolonged survival is as yet unavailable. However, it rapidly became clear that this was associated with high rates of disease recurrence. The results of hepatic transplantation for metastatic tumours are particularly poor. The two largest series describe 2-year survival rates between 14% and 19%, with 5-year survival rates not exceeding 5%. In 1989, the Pittsburgh group reported a series of five patients with neuroendocrine hepatic metastases, three of whom were alive at 7, 16 and 34 months following surgery. Five patients had died, four from recurrent disease and one from chronic rejection. In this study, tumour recurrence for patients with carcinoid tumours was more frequent. However, it was thought that this may have reflected the fact that transplantation was carried out later in the course of the disease process in carcinoid patients or due to differential effects of immunosuppression on tumour growth. One patient underwent upper abdominal exenteration with liver replacement for a large pancreatic tumour. With this exception, all patients had previously undergone resection of the primary tumour. In this series, despite extensive preoperative imaging, extrahepatic tumour was found in four patients and this was resected. Two patients died as a result of portal vein thrombosis and one patient died at day 7 from overwhelming septicaemia. Of the remaining patients, one patient died at 17 months as a result of bone and liver recurrence. Three patients were alive at 15, 24 and 62 months, but the longest survivor had developed bone and liver metastases. The authors have recommended that transplantation should only be offered to patients with symptomatic disease that has failed to respond to all other therapies. In addition, they conclude that the finding of extrahepatic disease at Management of neuroendocrine tumours 263 laparotomy should probably result in the abandoning of the transplant procedure. Two patients died from tumour recurrence, one at 6 months, the other at 5 years post-transplantation. Four of these patients were disease-free 2, 57, 58 and 103 months post-transplantation. All patients experienced good symptomatic relief and postoperative hormone levels were within normal ranges. The largest series of patients undergoing liver transplantation for the treatment of neuroendocrine hepatic metastases comes from France. Twelve patients subsequently died; four of these deaths were due to delayed technical or other non-tumour complications. All seven patients that had undergone upper abdominal exenteration died from immediate or delayed surgical complications. At the time of their publication, 13 patients were alive and in eight of these there was no evidence of recurrent disease. However, the survival rate for carcinoid tumours was significantly higher with a 5-year survival rate of 69%. This reflected a lower postoperative mortality for patients with carcinoid tumours and the fact that disease recurrence was more compatible with long-term survival. Overall survival figures are not dissimilar from the 25 to 35% 5-year survival reported for non-transplant treatments. There is now a broad consensus regarding the indications and timing of liver transplantation for patients with neuroendocrine hepatic metastases. This allows a full laparotomy to be performed and extrahepatic disease may be identified at this time. Inevitably some patients may present later with extrahepatic disease and then no longer be candidates for transplantation. However, transplantation continues to be associated with high surgical mortality and many patients can be maintained on medical therapy for a prolonged period of time. Finally, if extrahepatic disease is identified at the time of transplant, the procedure should probably be abandoned. Thus patients with symptomatic disease that have failed to respond to all other treatments may be considered candidates for hepatic transplantation. In selected patients transplantation offers good palliation and for a small proportion of patients there may be the possibility of cure. Medical treatment Chemotherapy Chemotherapy has only a very limited role to play in the treatment of patients with neuroendocrine hepatic metastases. For patients with endocrine pancreatic tumours, Surgical Management of hepatobiliary and pancreatic disorders 264 single agent chemotherapy generally has poor response rates of between 7 and 25%. The combination of streptozotocin and doxirubicin has been more effective, with tumour regression observed in up to 69% of patients.

Such skull fractures may occur in cases of precipitate delivery blood pressure guide purchase discount aldactone on-line, inappropriate use of forceps blood pressure medication list a-z order cheap aldactone on line, or prolonged labor with disproportion between the size of the fetal head and birth canal arteria hepatica propia buy aldactone with amex. Perinatal Infections Infections of the embryo blood pressure chart on age purchase 25mg aldactone, fetus, and neonate are manifested in a variety of ways and are mentioned as etiologic factors in numerous other sections within this chapter. Occasionally, infections occur by a combination of the two routes in that an ascending microorganism infects the endometrium and then the fetal bloodstream via the chorionic villi. In general, the fetus acquires the infection either by inhaling infected amniotic fluid into the lungs shortly before birth or by passing through an infected birth canal during delivery. As previously stated, preterm birth is often an unfortunate consequence and may be related either to damage and rupture of the amniotic sac as a direct consequence of the inflammation or to the induction of labor associated with a release of prostaglandins by the infiltrating neutrophils. Chorioamnionitis of the placental membranes and funisitis are usually demonstrable, although the presence or absence and severity of chorioamnionitis do not necessarily correlate with the severity of the fetal infection. In the fetus infected via inhalation of amniotic fluid, pneumonia, sepsis, and meningitis are the most common sequelae. The clinical manifestations of these infections are highly variable, depending largely on the gestational timing and microorganism involved. While the virus can bind to different cell types, replication occurs only in erythroid cells, and diagnostic viral cytopathic effect can be recognized in late erythroid progenitor cells of infected infants (Fig. Such infections occurring early in gestation may also cause chronic sequelae in the child, including growth and mental retardation, cataracts, congenital cardiac anomalies, and bone defects. Most cases of early-onset sepsis are acquired at or shortly before birth and tend to result in clinical signs and symptoms of pneumonia, sepsis, and occasionally meningitis within 4 or 5 days of life. Group B streptococcus is the most common Figure 10-9 Bone marrow from an infant infected with parvovirus B19. The arrows point to two erythroid precursors with large homogeneous intranuclear inclusions and a surrounding peripheral rim of residual chromatin. Figure 10-10 Schematic outline of the pathophysiology of the respiratory distress syndrome (see text). B, the congested portion of the ileum corresponds to areas of hemorrhagic infarction and transmural necrosis microscopically. Submucosal gas bubbles (pneumatosis intestinalis) can be seen in several areas (arrows). In B, fluid accumulation is particularly prominent in the soft tissues of the neck, and this condition has been termed cystic hygroma. Cystic hygromas are characteristically seen, but not limited to , constitutional chromosomal anomalies such as 45,X0 karyotypes. When the fetus inherits red cell antigenic determinants from the father that are foreign to the mother, a maternal immune reaction may occur, leading to hemolytic disease in the infant. The incidence of immune hydrops in urban populations has declined remarkably, owing largely to the current methods of preventing Rh immunization in at-risk mothers. Successful prophylaxis of this disorder has resulted directly from an understanding of its pathogenesis. The underlying basis of immune hydrops is the immunization of the mother by blood group antigens on fetal red cells and the free passage of antibodies from the mother through the placenta to the fetus (Fig. Fetal red cells may reach the maternal circulation during the last trimester of pregnancy, when the cytotrophoblast is no longer present as a barrier, or during childbirth itself. Of the numerous antigens included in the Rh system, only the D antigen is the major cause of Rh incompatibility. Figure 10-15 Numerous islands of extramedullary hematopoiesis (small blue cells) are scattered among mature hepatocytes in this infant with nonimmune hydrops fetalis. Severe hyperbilirubinemia in the neonatal period, for example, secondary to immune hemolysis, results in deposition of bilirubin pigment in the brain parenchyma. This occurs because the blood-brain barrier is less well developed in the neonatal period than it is in adulthood. Homozygotes with this autosomal recessive disorder classically have a severe deficiency of phenylalanine hydroxylase, leading to hyperphenylalaninemia and its pathologic consequences. Affected infants are normal at birth but within a few weeks develop a rising plasma phenylalanine level, which in some way impairs brain development. About one third of these children are never able to walk, and two thirds cannot talk. Seizures, other neurologic abnormalities, decreased pigmentation of hair and skin, and eczema often accompany the mental retardation in untreated children. Hyperphenylalaninemia and the resultant mental retardation can be avoided by restriction of phenylalanine intake early in life. Between 75% and 90% of children born to such women are mentally retarded and 488 microcephalic, and 15% have congenital heart disease, even though the infants themselves are heterozygotes. The presence and severity of the fetal anomalies directly correlate with the maternal phenylalanine level, so it is imperative that maternal dietary restriction of phenylalanine is initiated before conception and continues throughout the pregnancy. In normal children, less than 50% of the dietary intake of phenylalanine is necessary for protein synthesis. The rest is irreversibly converted to tyrosine by a complex hepatic phenylalanine hydroxylase system (Fig. Although neonatal hyperphenylalaninemia can be caused by deficiencies in any of these components, 98% to 99% of cases are attributable to abnormalities in phenylalanine hydroxylase. Some of these abnormal metabolites are excreted in the sweat, and phenylacetic acid in particular imparts a strong musty or mousy odor to affected infants. At the molecular level, several mutant alleles of the phenylalanine hydroxylase gene have been identified. Each mutation induces a particular alteration in the enzyme resulting in a corresponding quantitative effect on residual enzyme activity ranging from complete absence to 50% of normal values. The degree of hyperphenylalaninemia and clinical phenotype is inversely related to the amount of residual enzyme activity. Moreover, some mutations result in only modest elevations of phenylalanine levels, and the affected children have no neurologic damage. Figure 10-21 Chloride channel defect in the sweat duct (top) causes increased chloride and sodium concentration in sweat. In the airway (bottom), cystic fibrosis patients have decreased chloride secretion and increased sodium and water reabsorption leading to dehydration of the mucus layer coating epithelial cells, defective mucociliary action, and mucus plugging of airways. Figure 10-22 the many clinical manifestations of mutations in the cystic fibrosis gene, from most severe to asymptomatic. The ducts are dilated and plugged with eosinophilic mucin, and the parenchymal glands are atrophic and replaced by fibrous tissue. Nutritional: failure to thrive (protein-calorie malnutrition), hypoproteinemia, edema, complications secondary to fat-soluble vitamin deficiency 3. Approximately 5% to 10% of the cases come to clinical attention at birth or soon after because of an attack of meconium ileus. Distal intestinal obstruction can also occur in older individuals, manifesting as recurrent episodes of right lower quadrant pain sometimes associated with a palpable mass in the right iliac fossa. Pancreatic insufficiency is associated with protein and fat malabsorption and increased fecal loss. The faulty fat absorption may induce deficiency of the fat-soluble vitamins, resulting in manifestations of avitaminosis A, D, or K. Persistent diarrhea may result in rectal prolapse in up to 10% of children with cystic fibrosis. The pancreas sufficient phenotype is usually not associated with other gastrointestinal complications, and in general, these individuals demonstrate excellent growth and development. Cardiorespiratory complications, such as persistent lung infections, obstructive pulmonary disease, and cor pulmonale, are the single most common cause of death (80%) in patients in the [86] United States. With the indiscriminate use of antibiotic prophylaxis against Staphylococcus, there has been an unfortunate resurgence of resistant strains of Pseudomonas in many patients.

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