Summary Chronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11‐year‐old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.
Feeding and the consequences of feeding (growth and development) are central themes of pediatrics. How infants and toddlers feed and what they eat are affected by current fads, the media, and the family’s ethnic background. However, certain facts about feeding and nutrition remain constant despite cultural variations. The pediatrician or other clinician must be aware of these facts to educate and assist a family in the appropriate feeding of its child.Healthy term infants, whether breastfed or formula-fed, need about 95 to 115 kcal/kg per day during the first 6 months of life. Approximately 8% to 12% of calories should be provided by protein and about 30% to 50% by fat. If these requirements are met, a growing term infant can be expected to gain between 25 to 40 g/day in the first 3 months and about 15 to 20 g/day in the second 3 months.Caloric and protein requirements for preterm infants are greater than those for term babies. To attain good growth, a healthy preterm baby needs about 120 to 130 kcal/kg per day, including about 2.0 g/kg per day of human milk protein or 2. 5 to 3.0 g/kg per day of formula protein. These nutritional requirements can be met by adding an available fortifier to human milk, adjusting the milk to a caloric density of 24 kcal/oz, or using one of the proprietary formulas specially modified for preterm infants.The advent of commercially produced cow milk-based formulae has increased the exposure of infants to antigenic foreign proteins. Although variability in diagnostic criteria makes it difficult to ascertain the true prevalence of cow milk protein allergy, reports suggest that allergic reactions to cow milk occur in 0.3%to 7.5% of infants and toddlers. The clinical manifestations may include classic atopy (rhinitis, wheezing, atopic dermatitis), colitis(blood-streaked diarrhea), and anaphylaxis (vomiting, diarrhea, circulatory collapse). The diagnosis is made by giving an oral challenge with the antigen (in this case, cow milk protein) and observing for: 1) vomiting or diarrhea, 2) an increase in peripheral neutrophil count of 3,500/mm3 from pre- to postchallenge; 3) blood in the stool; 4) white blood cells in a Wright-stained stool; and 5) Charcot-Leyden crystals (eosinophil debris) in the stool. If three or more of these manifestations are present, the challenge is considered positive.A key point to remember is that soy protein’s reputation for being hypoallergenic is entirely undeserved. Most studies indicate that 40% to 50% of children allergic to cow milk protein also will develop allergy to soy protein. The practice of feeding soy-based formulae to children proven or suspected of being allergic to cow milk protein, therefore, is unwarranted. Instead, the use of a casein hydrolysate formula is much more appropriate: Hydrolyzing the antigenic proteins in cow milk eliminates their allergenicity. It also should be remembered that human milk contains foreign food proteins ingested by the mother; thus, a breastfed infant may have any of the allergic responses usually associated with formula feeding.Some infants may have feeding difficulties and/or delayed growth related to abnormalities of the gastrointestinal tract(swallowing, digestion, absorption, motility, or cholestasis) or to hypermetabolism. In some cases where maldigestion/malabsorption is the problem, special commercially produced formulae containing medium-chain triglycerides (MCT) and/or casein hydrolysate may be appropriate. In other cases, products can be added to standard formulae to increase their caloric density, an approach preferable to overconcentrating a formula by mixing with less than the usual amount of water. Although overconcentration increases the caloric density, it also raises both the gastrointestinal and renal solute loads. The two products used most frequently to increase the caloric density of a formula are MCT oil and glucose polymers. Unlike long-chain triglycerides, MCT oil is absorbed directly into the portal vein without needing to be solubilized by bile salts or digested by lipase, and it contains 115 kcal per tablespoon. However, MCT oil acts as a cathartic,and doses must be titrated so that abdominal pain, distension, and diarrhea can be avoided. MCT oil also can cause ketonemia, and when it is the predominant source of fat, it can lead to essential fatty acid deficiency. Other practical limitations to the use of MCT oil are its unpalatable taste and its expense. Glucose polymers, which are available as a liquid (30 kcal/tbs) or powder (23 kcal/tbs), increase calories without raising solute load. A danger with these additives is that if used in large amounts, they may reduce the nutrient density of proteins, minerals, and vitamins in the formula to inadequate levels.Finally, in this era of consumerism and health-consciousness, many parents again are preparing homemade baby foods rather than buying commercially available products. Commercial baby foods are safe, well-preserved, and free of the increased salt, monosodium glutamate, and nitrates that often appear in canned foods for adult consumption. Homemade baby foods are inexpensive and usually healthy and give parents great autonomy. When prepared at home, baby foods do not have to be sterile, but care must be taken to avoid contamination. Unused portions of food should be stored frozen in small servings and not kept for prolonged periods. Mothers often season baby foods to their own tastes rather than to the baby’s needs: Studies have shown that mother-prepared foods contain about ten times the salt found in commercial baby food and about 65% more than the maximum amount suggested by the National Academy of Sciences.Certain vegetables and fruits, such as spinach, carrots, beets,beans, and bananas, have high levels of nitrates, which are harmless. However, home preparation with subsequent storage allows the nitrates to convert to nitrites, which have been associated with methemoglobinemia in infants. If care and thoughtfulness are employed, both commercial and homemade baby foods can be safe and wholesome.Confusion between food allergy and food intolerance is common among the public and not so rare among pediatricians. An allergic response to cow milk formula is mediated by the immune system (frequently, but not exclusively, by IgE) in reaction to a protein antigen. As Dr Aiges points out, children who are allergic to cow milk protein frequently have a cross-sensitivity to protein in soy-based milks, making soy formula a potentially dangerous substitute. Lactose intolerance, on the other hand, is not an allergic phenomenon; it represents either a transient or permanent deficiency of lactase, the enzyme needed to metabolize lactose into its two constituent saccharides—galactose and glucose. When the lactose in cow milk cannot be broken down, it remains unabsorbed in the gut and may lead to bloating, abdominal discomfort,flatulence, and diarrhea. Soy formulas are free of lactose, instead containing sucrose and corn syrup as their carbohydrates. For children who have lactose intolerance, soy-based foods offer an appropriate alternative to dairy products.
SUMMARY This study was undertaken to determine if asymptomatic children and adolescents with inflammatory bowel disease and moderate to severe anorectosigmoid inflammation might remain symptom‐free for at least 12 months without specific intrarectal therapy. We prospectively studied 13 asymptomatic patients 6–21 years of age (four with Crohn's disease and nine with nonspecific colitis) with previously documented anorec‐tosigmoid inflammation. Of these 13, four had moderate to severe anorectosigmoid inflammation both endoscopi‐cally and histologically. These four patients (two with Crohn's disease and two with nonspecific colitis) were entered into the second phase of the study. Three were receiving sulfasalazine, and one received methylpredni‐solone, 4 mg/day, and 6‐mercaptopurine, 50 mg/day. None received intrarectal therapy. Clinical evaluation revealed that all four remained asymptomatic for 12 months despite the continued presence of moderate to severe anorectosigmoid inflammation. These results indicate that in children and adolescents with inflammatory bowel disease, the presence of inflammation of the anorectosigmoid does not necessarily correlate with or presage the onset of symptoms of proctosigmoiditis. Therefore, active inflammation of the anorectosigmoid is not the sole prerequisite for intrarectal therapy. The clinician should be guided by the symptoms of the patient, not by the presence or absence of active anorectosigmoid inflammation.
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Five children were noted to have arteriohepatic dysplasia (Alagille's syndrome) between 3 and 7 months of age. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangiograms failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated with fibrosis and increased vascularity. Hypoplasia of the gallbladder occurred in two patients. Changes were observed in the porta hepatis. Eighty of 208 micrometers bile ducts were associated with peripherally located gland-like structures. These changes are indistinguishable from those in fibrous remnants of extrahepatic biliary atresia. Hepatic features of sequential liver biopsies obtained in the five patients were divided into early and late changes. From birth to 3 months of age, the pathology consisted of cholestasis and bile duct destruction. After 3 months of age, there was persistent cholestasis, paucity of interlobular bile ducts, and portal fibrosis. Ductular proliferation was not an intrinsic change. When present, it was related to a recent episode of cholangitis.
Paucity of interlobular bile ducts is a common feature of cholestatic liver disease in premature infants. Whereas a bile duct to portal space ratio of 0.9 to 1.8 is cited by Alagille as the norm for children, there are no data regarding the normal bile duct to portal space ratio in preterm infants. In this study, by examining liver tissue obtained from autopsied fetuses and infants, we have determined that the bile duct to portal space ratio increases as a function of postconceptional age. After 38 weeks postconception, a ratio equal to or greater than 0.9 was seen in eight of nine subjects. Therefore, in evaluating premature infants for duct paucity, liver biopsies should be obtained only after 38 weeks. Prior to 38 weeks postconception, a bile duct to portal space ratio less than 0.9 may be physiologic.
Sulfasalazine (SASP) is frequently used in the treatment of chronic inflammatory bowel disease (IBD), particularly colitis. Because the drug poses a theoretical risk for renal tubular damage, 26 patients, 8–18 years of age, with Crohn's ileocolitis were studied. Thirteen children were receiving SASP while 13 served as disease controls. Renal tubular function was assessed by measurement of urinary β 2 ‐microglobulin and n ‐acetylglucosa‐minidase activity. No abnormalities were found on routine measurement of renal function. Similarly, urinary β 2 ‐microglobulin and n ‐acetylglucosaminidase activities were within normal limits for patients receiving SASP, as well as for disease controls. Although there is a theoretical risk for renal tubular damage from the prolonged use of SASP, this study would suggest that IBD patients receiving the drug are at no greater risk for renal injury than their counterparts not receiving the medication.
