Pediatrics
Board Review Online

Pseudotumor cerebri is a syndrome of increased intracranial pressure with normal cell and protein count on lumbar puncture and normal ventricular size and anatomy on MRI. Symptoms include headache with possible vomiting and diplopia. On examination, infants typically present with a bulging fontanel, while older patients may have papilledema.

Risks for pseudotumor cerebri include conditions causing changes in cerebrospinal fluid absorption and production, cerebral edema, changes in cerebral blood flow, and venous obstruction. These include metabolic causes, infections, drugs, hematologic disorders, and head injury. When no cause is found, the condition is called idiopathic intracranial hypertension.

References:
Pless ML. Pseudotumor cerebri. In: Kliegman RM, et al, eds. Nelson Textbook of Pediatrics. 19th ed., 2011:2099-2100.

A microcolon indicative of CF and meconium ileus would be expected results on a barium enema. CF is an autosomal recessive disorder caused by mutations of both alleles of the large, 250,000-base-pair gene located on chromosome number 7 called CFTR. The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a chloride channel regulated by cyclic adenosine monophosphate and usually resides within the apical portion of mucosal epithelial cells.

CF screening using IRT is now a routine part of newborn screening. IRT is a measurable fraction of pancreatic enzymes that normally is released at extremely low concentrations into the systemic circulation. Results of newborn screening for CF are considered positive when IRT concentrations in the serum elevated.

 

Absent or impaired CFTR chloride transport in CF often leads to pathologic intestinal obstruction due to marked thickening of intestinal secretions. This event frequently is manifested as meconium ileus in newborns. Affected infants fail to pass stool within the first few days after birth and may develop symptoms of acute intestinal obstruction and visceral perforation. Symptoms of meconium ileus include emesis that may be bilious, abdominal distention, and failure to pass meconium within the first 12 to 24 hours. Diagnosis is based on clinical presentation and diagnostic radiology.

Obstruction occurs at the level of the terminal ileum (unlike the colonic obstruction caused by meconium-plug syndrome). A barium enema will reveal obstruction at the terminal ileum and a microcolon, distal to the obstruction, and a relatively narrow, empty, small-caliber colon. Abdominal x-rays will show dilated intestinal loops and a have a "soap bubble" or "ground glass" appearance due to small air bubbles mixed with meconium, which is diagnostic of meconium ileus.

Treatment involves enemas with dilute contrast and, if enemas fail, then surgery will be necessary

Jejunoileal atresia may present with similar clinical symptoms and a microcolon but will have a "triple bubble" appearance on abdominal imaging.

References:

Cochrane WJ, ed. Meconium ileus. In: Porter RS, et al, eds. Merck Manual. 2013.

Lahiri T, Hempstead SE, Brady C, et al. Clinical practice guidelines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis. Pediatrics. 2016. Epub ahead of print.

Montgomery GS, Howenstine M. Cystic fibrosis. Pediatr Rev. 2009;30(8):302-309.

Penicillin is the treatment of choice in order to prevent rheumatic fever and other sequelae. The incidence of poststreptococcal glomerulonephritis does not appear to be significantly reduced. Necrotizing fasciitis is not a common complication. A patient should be kept home for 24 hours after the start of antibiotics to prevent transmission.

Smoking cigarettes and consuming nicotine increase the resting heart rate as soon as 30 minutes after inhaling; the higher the nicotine consumption, the higher the heart rate.

Although not all people who smoke have asthma, they are susceptible to bronchoconstriction from tobacco smoke. A person with asthma who starts smoking can worsen his or her condition.

Smoking and nicotine exposure cause vasoconstriction by two mechanisms:  1) decreasing nitric oxide, , and 2) increasing endothelin 1. The net effect is constriction of the blood vessels immediately following smoking. Nicotine consumption also increases blood pressure. People who smoke have a higher systolic blood pressure than those who do not smoke.

References:

Committee on Environmental Health; Committee on Substance Abuse; Committee on Adolescence; Committee on Native American Child. Policy statement—tobacco use: a pediatric disease. Pediatrics. 2009;124(5):1474-1487.

Doe J, DeSanto C, Granger D, et al. Smoking's immediate effects on the body. Published September 2009. Accessed June 1, 2015.

This infant should be assessed for vitamin A deficiency. This case represents classic cystic fibrosis (CF) with failure to thrive. Children with this malabsorption condition are at risk for deficiencies of fat-soluble vitamins, particularly vitamin A. Vitamin A deficiency is associated with poor vision, dry eyes, anemia, and corneal clouding.

Vitamin C is a water-soluble vitamin and deficiency is not typical in CF. Deficiency of vitamin C leads to bleeding in the joints and of the mucus membranes.

Hypocortisolemia is a hallmark of adrenal or pituitary insufficiency, but not cystic fibrosis.

Insulin deficiency is the basic abnormality seen in insulin-dependent diabetes. This entity can also be associated with failure to thrive, but is unusual in this age group, and is associated with watery, osmotic diarrhea, polyuria, and change in consciousness.

Reference:

Sabella C, Cunningham RJ III, eds. Cleveland Clinic Intensive Review of Pediatrics. 4th ed., 2013.