A Lange Medical Book Pediatrics on call - part 5 ppt

66. METABOLIC DISEASES 299 hypoplasia (complete or partial agenesis of the cerebellar vermis). Hydrocephalus can develop in utero or during early childhood (typi- cally, first year of life). In older children, the disorder can present with signs of increased intracranial pressure (lethargy, headache, and vomiting) or cerebellar dysfunction (ataxia). Other CNS anomalies may be present, including agenesis of the corpus callosum, hetero- topias, congenital tumors, and aqueductal stenosis. REFERENCES Bernard JP, Moscoso G, Renier D, Ville Y. Cystic malformation of the posterior fossa. Prenat Diagn 2001;21:1064–1069. Niesen CE. Malformations of the posterior fossa: Current perspectives. Sem Pediatr Neurol 2002;9:320–334. Pascual-Casatroviejo I, Velez A, Pascual-Pascual S, et al. Dandy-Walker malformation: Analysis of 38 cases. Child Nerv Sys 1991;7:88–97. 66. METABOLIC DISEASES I. Problem. A full-term neonate, who previously appeared well, pres- ents with rapidly increasing lethargy after 3–4 days of poor feeding. II. Immediate Questions A. Is there a family history of neonatal losses? Such a history is highly suspicious for metabolic disease caused by enzyme defi- ciencies. These diseases typically are transmitted in an autoso- mal-recessive or occasionally X-linked fashion, making the recurrence risk for these families significant. B. Is there associated vomiting? Can be nonspecific, or excessive with hyperammonemia. C. Does patient have an unusual odor? Organic acids are volatile and thus can be associated with an unusual odor of sweat, urine, or earwax. Maple syrup urine disease (MSUD) is often suspected from sweet-smelling earwax. A foul “sweaty feet” or “cat urine” odor can occur in several of the organic acidemias. D. If available, what were the newborn screening results? Newborn screening studies in many states include many of the organic acidemias, fatty acid oxidation defects, and urea cycle defects. Check screening results of child or other family member, if available. Because of limitations of screening tests, a negative study cannot be relied on to rule out disease (specifically, several urea cycle defects and energy production defects). Due to resid- ual enzyme function, a sample obtained before the onset of symp- toms may be normal, even in an affected patient. Abnormal results must be verified with acute samples (see later discussion under V, Plan). III. Differential Diagnosis A. Sepsis. Always consider in acutely ill or febrile neonates. Conversely, metabolic diseases are probably as common as true 300 I: ON CALL PROBLEMS sepsis and should be considered in all acutely sick neonates. In all suspicious cases, obtain appropriate cultures (eg, blood, cere- brospinal fluid, and urine) and consider appropriate antibiotics. Because infection can exacerbate metabolic disease, it should be considered even when a metabolic disorder is likely. B. Organic Acidemia. Most often caused by enzyme or cofactor deficiencies in the catabolism of branched chain amino acids (valine, leucine, and isoleucine). Organic acid and a positive gap acidosis develop from metabolites built up behind the enzymatic block. Other effects of metabolite excess include inhibition of enzymes of the urea cycle with secondary hyperammonemia. Many of these metabolites have direct CNS toxicity. Marrow sup- pression and altered glucose metabolism (hyperglycemia or hypoglycemia) also can occur as secondary effects. C. Primary Urea Cycle Defects. Typically result in hyperammone- mia without acidosis. Hallmark of these disorders is respiratory alkalosis in an ill-appearing child; hyperammonemia affects the respiratory centers, causing deep and rapid breathing (hyperp- nea) with resultant drop in carbon dioxide. D. Disorders Involving Energy Production 1. Glycogen storage disorders. Patients classically present with enlarged liver and subsequent preprandial hypoglycemia and may manifest acute hypoglycemia with intercurrent illness or fast. Lactic acidosis from the chronic energy depletion state provides a source of energy for the brain, and often the hypo- glycemia goes unnoticed until an illness occurs. Long-term sequelae can include liver adenomas, progressive renal insuf- ficiency, and gout. 2. Fatty acid oxidation (FAO) defects. Involve enzymatic defects in fatty acid ␤-oxidation. In fasting states, when glyco- gen stores are depleted, fats must be mobilized for energy pro- duction. If ␤-oxidation is impaired, hypoglycemia develops with relative hypoketosis or aketosis. Metabolites are organic acids, with resultant positive gap acidosis, and may have a direct toxic effect on the CNS. Myopathy, cardiomyopathy, retinopathy, and other systemic manifestations can occur over time in some patients with FAO defects. 3. Primary lactic acidosis. Typically thought of as disorders of gluconeogenesis, Krebs cycle, or the electron transport chain. Patients often present acutely with positive gap acidosis due to lactate or pyruvate, or both. Hypoglycemia is variable. E. Structural cardiac defects. Suggested by history and physical exam. F. Trauma. Altered mental status or vomiting can occur with head or abdominal trauma. G. Toxic Exposure (eg, organophosphates). H. Dehydration. From intercurrent GI illness or formula intolerance. 66. METABOLIC DISEASES 301 IV. Database A. Physical Exam Key Points 1. Vital signs. Tachypnea is a common reaction to stress in the neonate. When acidosis or hyperammonemia, or both, are present, hyperpnea (deep and rapid breathing) is often seen. Fever suggests infection, which can occur as a primary or sec- ondary phenomenon in metabolic disease. 2. HEENT. A full fontanelle can accompany meningitis and hyper- ammonemia (secondary to cerebral edema). Altered pupillary reactions with subsequent herniation can occur if untreated. If cataracts are present, consider galactosemia. Dry mucous membranes can indicate dehydration from poor feeding of any etiology. 3. Abdomen. Transient hepatomegaly can accompany many of the metabolic disorders. It is typical in disorders of energy pro- duction (eg, FAO defects and disorders of gluconeogenesis), resolving when metabolic stability is attained. Progressive hepatomegaly can be seen in the glycogen storage disorders. 4. Neurologic exam. Mental status changes are a common find- ing in neonates in distress. Metabolic considerations include hypoglycemia, hyperammonemia, and severe acidosis. Hyperreflexia and clonus can result from hyperammonemia- induced cerebral edema. B. Laboratory Data 1. Glucose a. Hypoglycemia. Ketotic hypoglycemia is seen in endocrine disorders, some organic acidemias, primary lactic acidoses, and some glycogen storage diseases. Hypoketotic or ake- totic hypoglycemia is seen in hyperinsulinism. Hypoglycemia is seen in metabolic disorders, including type I (“classic”) glycogen storage disease (Von Gierke), and is the hallmark of FAO defects. When considering hypoglycemia due to energy production disorders, the length of the fast may be helpful: glycogen is a fuel that is necessary shortly after meals (∼3–4 hours); fatty acid metabolism is the next oblig- atory fuel (∼4–8 hours); and gluconeogenesis is utilized thereafter. Prolonged fast or intercurrent vomiting and diar- rheal illness is typical of hypoglycemia with FAO defects; whereas a short fast (3–4 hours) may result in hypoglycemia in patients with glycogen storage disease. Fasting tolerance increases with age. b. Secondary hyperglycemia. Can also accompany organic acidemias. Ketosis may be seen in these disorders as well, making the presentation difficult to distinguish from neonatal diabetic ketoacidosis. 2. Urine ketones. Neonates make and use ketones highly effi- ciently, so they are a rare finding before 2–3 months of age. 302 I: ON CALL PROBLEMS Ketosis in a neonate suggests an organic acidemia. Outside of the neonatal period, inappropriate ketones in the face of a normal or elevated blood glucose level suggests organic acidemia. Conversely, absence of ketones in a hypoglycemic child suggests glycogen storage disease and FAO defects. See earlier discussion. 3. Electrolytes. Low bicarbonate suggests acidosis. ABGs should be obtained to confirm this, because hyperpnea caused by hyperammonemia can result in hypocarbia and compensa- tory renal wasting of bicarbonate. 4. ABGs. Metabolic acidosis is typically seen in acutely ill neonates, often due to lactic acidosis with respiratory or circu- latory compromise. Organic acidemias or lactic acidosis from metabolic disease should be considered. Respiratory alkalosis is unusual in an acutely ill child and is typical of the primary urea cycle defects (see III, C, 3, earlier). 5. Anion gap. Calculated as follows: Na − (Cl + HCO 3 ); normal anion gap is 12–15. In confirmed acidosis, an elevated anion gap is seen the presence of an unmeasured ion, such as an organic acid, lactate, excessive ketones, or toxic ingestion. 6. Blood ammonia. Typically significantly elevated in primary urea cycle defects. May be secondarily elevated in organic acidemias. Mild to modest elevations can be seen in FAO defects or primary lactic acidosis. 7. CBC. Elevated WBC count can suggest infection. Bone marrow suppression can occur in some organic acidemias and severe infections. 8. Liver function tests. May be elevated in many metabolic disorders (see IV, A, 3, earlier). 9. BUN. In urea cycle disturbances (primary or secondary), patients are unable to make urea; therefore, BUN is low even in the presence of dehydration. 10. Lactic acid. Can be elevated in tissue hypoxia from sepsis, seizure, and trauma. Often excessive in mitochondrial disease, primary lactic acidoses, and glycogen storage diseases. 11. Pyruvate. Lactate and pyruvate are in equilibrium, depending on the redox potential of the cell. In lactic acidosis, pyruvate eleva- tions and lactate-to-pyruvate ratios may help to localize the enzy- matic defect. These levels should be obtained simultaneously. 12. Uric acid. May be elevated in energy-deficient states such as the primary lactic acidoses, FAO defects, and glycogen stor- age diseases. Often excessive in glycogen storage diseases due to both overproduction and underexcretion. C. Radiographic and Other Studies 1. MRI and CT scans. May show evidence of cerebral edema when hyperammonemia is present. 2. Abdominal ultrasound. Microvesicular fatty infiltration is con- sistent with FAO defect. Hepatomegaly due to glycogen storage 66. METABOLIC DISEASES 303 is nonspecific on ultrasound; biopsy is usually required to iden- tify glycogen. V. Plan. Exact diagnosis may be made after the initial presentation using specimens obtained acutely. Such studies, including urine organic acids, plasma amino acids, and acylcarnitine profile, require specialized laboratories. For practical purposes, samples of acute urine and plasma (with cellular portion spun off and discarded) can be frozen and remain stable for days to months. If other samples cannot be obtained, a newborn screening filter paper dotted with blood and air-dried can be most helpful. Because some children with metabolic diseases appear biochemically normal when well, obtain- ing samples acutely is critical in establishing a diagnosis. A. Hemodialysis. For extreme acidosis or hyperammonemia with mental status changes, hemodialysis is the fastest method of ammonia removal. If medical center does not have this capability, emergent transfer is recommended. When cerebral edema is present, mannitol, hyperventilation, and ventilatory support may be used if herniation is suspected or impending. Correcting ammonia level and removing organic academia will resolve the cerebral edema in most situations. B. Stop Offending Agent. In primary urea cycle defects and the common organic acidemias, stopping protein intake is essential. C. Intravenous Dextrose. Essential in acute treatment of hypo- glycemia of any etiology. Ensuring a constant source of glucose until an appropriate diet can be established for FAO defects and glycogen storage disorders can prevent further hypoglycemic episodes. Providing an energy source to stop catabolism can pre- vent worsening of the clinical status in disorders involving protein metabolism (urea cycle disorders and organic acidemias). For neonates, 8–10 mg/kg/min, and for children, 6–8 mg/kg/min of IV dextrose is recommended. In organic acidemias, FAO defects, and primary urea cycle defects, a forced diuresis may help to rid the body of toxic metabolites, which are excreted in the kidneys. If a central line has not yet been established or in children with known metabolic disease presenting with acute exacerbations but without significant mental status changes, D 10 at 2 times maintenance with appropriate electrolytes may suffice. This treatment is appropriate in all of the common metabolic disorders, with the exception of the primary lactic acidemias and mitochondrial disease, because excess glucose may increase lactate production. Use dextrose cautiously with appropriate fluid hydration in these situations. D. Ammonia Scavengers. Sodium phenylbutyrate and sodium phenylacetate provide a route for ammonia removal in primary urea cycle disorders.These are orphan drugs and should be used only with the help of a metabolic specialist. E. Insulin. With large amounts of glucose used to stop catabolism, patients may develop hyperglycemia and associated fluid losses. 304 I: ON CALL PROBLEMS Hyperglycemia may be a presenting feature of some organic acidemias. To ensure that glucose given is being used to stop or prevent catabolism and promote anabolism, an insulin drip may be used. Insulin and growth hormone have both been used to promote anabolism in patients who are not responding to the usual measures. F. Vitamin or Cofactor Therapy. Until a diagnosis is established, treatment with cofactors for the most likely enzymes can be ben- eficial. Biotin, vitamin B 12 , is the cofactor most likely involved in the organic acidemias; thiamine and biotin in the primary lactic acidemias. MSUD, which is often apparent due to the typical odor, may respond to thiamine. G. L-Carnitine. Rarely available or used acutely. Provides a method of organic acid removal via esterification and renal clearance. Supplementation prevents a secondary carnitine deficiency. Caution should be used in treating certain FAO defects. H. Transfusion. For marrow suppression or excessive blood loss. Concern always exists of increasing the protein load in patients with disorders of protein metabolism. Usually well-tolerated, but monitor closely. I. Albumin and Fluid Resuscitation. Concern always exists of increasing the protein load in patients with disorders of protein metabolism. Although patients should be closely monitored, when necessary, this treatment is usually well-tolerated. J. Unexplained Death. In the case of potential metabolic disease, certain specimens may be most helpful in establishing a post- mortem diagnosis. Most of these disorders are autosomal reces- sive and thus pose a significant recurrence risk to families. Acute samples of plasma and urine may be sent for metabolic studies if kept frozen. A filter paper sample can provide metabolic informa- tion and is also a very stable source of DNA for future studies such as mutation analysis. If possible, premorten or immediate postmortem biopsy specimens from liver and muscle, flash frozen and stored at −40°C, may be used for enzyme analysis, DNA, and so forth. A fibroblast line established from a skin biopsy sample obtained using sterile technique premortem or immediately post- mortem may be used similarly, although at the current time, not all enzymes can be studied in fibroblasts. Samples can be placed in sterile saline and refrigerated until proper medium for culture can be obtained. VI. Problem Case Diagnosis. Physical exam of this term neonate was unremarkable. Laboratory workup was significant for hypoglycemia, acidosis, ketosis, and hyperammonemia. Results of bacterial cul- tures were negative. Diagnosis is methylmalonic acidemia. VII. Teaching Pearl: Question. What is the most likely cause of illness in an 11-month-old, previously healthy infant with a 3-day history of symp- toms of upper respiratory infection, diarrhea of 24 hours’ duration, and 67. NASOGASTRIC TUBE MANAGEMENT 305 poor oral intake? Liver is palpable on exam. Electrolytes are as fol- lows: Na 140, K 5.0, Cl 106, T CO2 12. Glucose level is 23 mg/dL. VBGs show pH of 7.29 and CO 2 of 28. AST is 112 and ALT, 86. Urinalysis shows 1+ ketonuria. VIII. Teaching Pearl: Answer. FAO defect; medium-chain acyl CoA dehy- drogenase (MCAD) is the most common of these defects and the most likely to present in a previously healthy child without other sys- tem involvement. REFERENCES Fernandes J, Saudubray J-M, van den Berghe G, eds. Inborn Metabolic Diseases: Diagnosis and Treatment, 3rd ed. Springer, 2000. Rimoin DL, Connor JM, Pyeritz Re, Korf BR, eds. Emery and Rimoin’s Principles and Practice of Medical Genetics, 4th ed. Churchill Livingstone, 2002. Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic & Molecular Basis of Inherited Disease, 8th ed. McGraw-Hill, 2001. 67. NASOGASTRIC TUBE MANAGEMENT I. Problem. A 3-year-old boy has bloody output from his nasogastric (NG) tube 2 days after undergoing small bowel resection for intus- susception. II. Immediate Questions A. What are the vital signs? Hypotension and tachycardia, in the presence of bleeding, are indicative of volume loss that requires prompt correction. B. What is the character of the NG bleeding? Lightly blood-tinged fluid or “coffee-ground” emesis is less worrisome than fresh red blood. C. How much bloody drainage has there been? Large amounts of bloody drainage are of concern. Blood volume in children aged 1–3 years is approximately 75 mL/kg. D. Has patient had recent or remote GI surgery? If surgery was recent, there may be bleeding from a new anastomotic site, or there may be a marginal ulcer at an old anastomotic site. E. Is patient passing flatus or stool? What is the character of the stools? Often, decreased NG output correlates with return of bowel function. Abdominal obstruction or ileus may result in decreased passage of gas or bowel movements. Fresh red blood from the rectum along with bloody NG drainage is very serious. Melena suggests upper tract or small bowel bleeding. Stools that are normal in appearance and occult blood–positive are sugges- tive of slower GI bleeding. Stools that are negative for occult blood suggest very early or insignificant bleeding. F. How long has NG tube been in place? A tube that has been recently placed may have a small amount of bloody drainage secondary to the insertion. A tube that has been in place for 306 I: ON CALL PROBLEMS > 48 hours may cause oozing from gastric or esophageal mucosal irritation. G. Is patient receiving antacids, H 2 blockers, or proton pump inhibitors (PPIs)? What is the pH of the drainage? Presence of acidic gastric secretions may predispose to formation of gas- tritis and “stress” ulcers. Gastric pH > 4 may enhance mucosal protection. H. Is there associated abdominal distention? If patient develops ileus or obstruction, the amount of aspirate may increase. I. Is output bilious? Bilious NG output indicates bile reflux into the stomach, or NG tube that has been placed distal to the pylorus. J. Is tube functioning? Tubes often become obstructed with mucous or medications. While the tube is on suction, listen for a whistle, which indicates patency. K. Is patient taking, or being given, extra fluid by mouth? Often, excessive amounts of ice chips are given to patients with NG tubes. This can lead to high NG outputs. Careful questioning of family and caregivers can identify this possibility. L. Are there any respiratory symptoms? If NG tube is misplaced in the esophagus or oropharynx, patient may have a cough or complain of throat pain. III. Differential Diagnosis A. Bloody NG Drainage 1. Insertion trauma. Usually nasopharyngeal. 2. Mucosal irritation. Often results from a tube that has been in place for > 48 hours; there is usually an associated acidic pH. 3. Swallowed pharyngeal blood. Posterior nosebleeds may not be clinically obvious. 4. Suture line disruption or hemorrhage. More likely in a patient who has had recent surgery. 5. Gastric or duodenal ulceration. More common in severely ill patients (eg, premature infants, burns, sepsis, head injury, steroid use, pancreatitis). 6. Gastric erosion or gastritis, esophagitis or Mallory-Weiss tear, esophageal varices. Mallory-Weiss tears are more fre- quent in patients who have had forceful vomiting or retching. Esophageal varices can result in severe GI bleeding. 7. Aortoenteric fistula. Severe GI bleeding; may be secondary to foreign body ingestion or occur after aortic surgery. 8. Coagulopathy. B. Change in Output of NG Drainage 1. Increased output a. Tip of tube distal to pylorus. NG tube aspirates all biliary and pancreatic solutions, as well as gastric output. b. Gastric outlet or small bowel obstruction. NG tube can irri- tate the pylorus and create edema or a pyloric channel ulcer. 67. NASOGASTRIC TUBE MANAGEMENT 307 c. Surreptitious fluid ingestion. 2. Decreased output a. Return of normal bowel motility and function. b. Obstructed or kinked tube. c. Medications. Agents that improve motility and gastric emp- tying, such as metoclopramide. d. Tip of tube in esophagus. Above the GE junction or coiled in the esophagus. IV. Database A. Physical Exam Key Points 1. Vital signs. Tachycardia, hypotension, hypoxemia, and fever are suggestive of substantial bleeding or sepsis, or both. 2. Mouth. Check that tube is not kinked in the mouth or throat. Look for evidence of oral, nasal, or pharyngeal bleeding. 3. Abdomen. Look for distention, tenderness, and peritoneal signs. Listen for bowel sounds. Absence of bowel sounds indi- cates obstruction. Distention occurs with ileus or obstruction. 4. Rectal exam. Is stool present? Absence of stool may reflect an anatomic obstruction. Check stool for occult blood. Assess color and character of stool (normal versus melena versus fresh blood). 5. Tube. Check patency and function by flushing with air or water. Check gastric fluid pH if tube is patent; pH < 4 promotes bleeding. B. Laboratory Data 1. CBC with platelets. Check for anemia as well as evidence of inflammation or infection. 2. PT and PTT. Evaluates clotting ability. 3. Type and crossmatch. For significant bleeding. 4. Amylase and lipase. Screen for pancreatitis. 5. Blood cultures. For fever, tachycardia. 6. Serum electrolytes. Carefully monitor patient’s hydration, as well as potassium and bicarbonate levels, during continuous suction. 7. NG aspirate. A pH > 6 indicates use of antacids or H 2 blockers or that tip of the tube is distal to the pylorus. C. Radiographic and Other Studies 1. Chest x-ray and abdominal obstruction series. Look for free intraperitoneal air or obstruction. Mediastinal air suggests esophageal perforation. Upright chest x-ray may show a large stomach bubble, indicating poor gastric emptying. Check posi- tion of the tube. Upright and flat abdominal x-rays may show distended bowel, indicating ileus or obstruction. 2. Contrast swallow study. To identify gastric outlet obstruction or partial small bowel obstruction, order a Gastrografin or dilute barium swallow study. Contrast should not be used in patients with ileus or complete obstruction. [...]... therapy should target Hib Use third-generation cephalosporin or ampicillin-sulbactam because of increasing ampicillin resistance of H influenzae S aureus, S pneumoniae, and GABHS are occasionally isolated Bacterial Tracheitis Endotracheal intubation is frequently necessary, and antibiotic therapy should target S aureus, GABHS, Moraxella catarrhalis, and S pneumoniae VI Problem Case Diagnosis On physical... throat, and tender cervical adenopathy Headache, nausea, vomiting, and abdominal pain are common Marked erythema of throat is present, with hyperemic, exudative tonsils and palatal petechiae Nasal congestion and rhinorrhea is usually absent More common in late winter and early spring 2 Epstein-Barr virus (EBV) Can cause severe exudative pharyngitis with fever, palatal petechiae, posterior cervical lymphadenopathy,... antidepressants Used for analgesia and sleep in chronic pain situations; not for acute pain management 3 Anticonvulsants Carbamazepine and gabapentin; used for neuropathic pain 4 Anxiolytics Benzodiazepines; use for anxiety and not as a substitute for opioids 5 Other medications Stimulants, SSRIs, steroids, topical patches, and ␣2-blockers are valuable adjuvants in many situations, but not in acute pain 6... stimulation of intercostal nerves or nerves in parietal pleura; diaphragmatic irritation; subdiaphragmatic abscess; bone infarction associated with acute chest syndrome of sickle cell disease; and leukemic infiltration of ribs and sternum b Lungs Pneumothorax; pleural effusion associated with lobar pneumonia, tuberculosis, or small pulmonary embolism; pulmonary infarction; pulmonary laceration; tracheal... lymphadenopathy, periorbital edema, and splenomegaly Coinfection with GABHS is common 3 Adenovirus Commonly causes exudative pharyngitis or pharyngoconjunctival fever Ipsilateral preauricular adenopathy is a helpful clue to diagnosis 4 Coxsackievirus Typically occurs in summer and early fall Causes herpangina with multiple small vesicles on tonsils and soft palate Coxsackievirus A1 6 causes so-called hand-footmouth... in these cases Neonatal torticollis Treatment includes range-of-motion exercises and other physical therapy If facial asymmetry occurs, surgical intervention may be necessary Lymphoma (Hodgkin and non-Hodgkin), rhabdomyosarcoma, and other malignant tumors Consultation with a pediatric oncologist is necessary for management and treatment of these diagnoses Uncommon diagnoses Ludwig angina and Lemierre... causes dysphagia Palatal petechiae are often associated with GABHS and EBV Coxsackievirus often causes small ulcers on soft palate and buccal mucosa HSV causes vesicles and ulcers on lips and gingival mucosa Asymmetric tonsillar enlargement and a deviated uvula are present with peritonsillar abscesses Posterior pharyngeal fullness and fluctuance may be noted with retropharyngeal abscess Trismus may be noted... peritonsillar abscess 4 Skin Sandpaper or scarlatiniform rash may be noted with GABHS scarlet fever Nonspecific morbilliform rash can occur after amoxicillin is given to a patient with EBV Erythroderma may signify toxin-mediated disease 328 I: ON CALL PROBLEMS 5 Abdomen Splenomegaly is associated with EBV B Laboratory Data 1 Rapid streptococcal antigen and throat culture Perform on patients with a stable... Epidural analgesia Specialized technique, and only indicated when pain is severe and refractory to oral and parenteral analgesics F Nonpharmacologic Modalities These methods can be very important in long-term management of recurring pain in conditions such as sickle cell disease Include behavioral (eg, biofeedback, deep breathing), psychological (eg, distraction, hypnosis education), and physical (hydration,... infection? Inflammation secondary to pulmonary infection can cause pneumothorax Does patient smoke cigarettes? Airway inflammation associated with cigarette smoking is accompanied by an increased incidence of pneumothorax If female, does patient have her menses? A pneumothorax associated with the beginning of the menstrual flow is known as catamenial pneumothorax III Differential Diagnosis Acute chest pain . CE. Malformations of the posterior fossa: Current perspectives. Sem Pediatr Neurol 2002;9:320–334. Pascual-Casatroviejo I, Velez A, Pascual-Pascual S, et al. Dandy-Walker malformation: Analysis. phenomenon in metabolic disease. 2. HEENT. A full fontanelle can accompany meningitis and hyper- ammonemia (secondary to cerebral edema). Altered pupillary reactions with subsequent herniation can. this, because hyperpnea caused by hyperammonemia can result in hypocarbia and compensa- tory renal wasting of bicarbonate. 4. ABGs. Metabolic acidosis is typically seen in acutely ill neonates,