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Chronic Granulomatous Disease of Childhood

Chronic granulomatous disease results from an inability of phagocytic cells to produce bactericidal superoxide anions caused by a defect in the nicotinamide adenine dinucleotide phosphate (reduced form) oxidase enzyme of phagocytes. This leads to recurrent life-threatening bacterial and fungal infections. About two thirds of patients inherit

Gastritis Cardia
Fig. 3.15. Eosinophic gastritis: thickened mucosal folds and a suggestion of nodularity in a patient with diarrhea and eosinophilia. (Courtesy of Dr. Carlo Buonomo, Boston, MA, USA)

X-linked defects; one-third inherit this disease in autosomal recessive fashion. Over 75% of patients present during the first 5 years of life. The most commonly involved organs are those that serve as barriers against the entry of microorganisms from the environment (e.g. lymph nodes, lungs, skin, GI tract, skeleton, upper respiratory tract and central nervous system) (MovAHEdi et al. 2004; Garcia-EuLAtE et al. 2006).

Patients with GI tract involvement will present with abdominal pain (periumbilical or diffuse) with lower abdominal cramping, diarrhea with or without blood in the stool, nausea and vomiting, constipation growth delay or hypoalbuminemia (Marciano et al. 2004).

A barium study (Fig. 3.16) will demonstrate narrowing of the antropyloric region (occurring in up to 16% of patients) (Griscom et al. 1974) secondary to chronic inflammation and fibrosis, which can eventually lead to gastric outlet obstruction. In some cases, the proximal duodenum will also be involved. Ultrasound will also reveal a thickening of the antro-pyloric wall, simulating HPS. Most patients, however, will be older than the typical HPS patient.

Fig. 3.16. a CGDC stomach. Antral narrowing in a 7-year-old with CGDC. b Mid-esophageal stenosis caused by enlarged lymph nodes in CGDC

Fig. 3.16. a CGDC stomach. Antral narrowing in a 7-year-old with CGDC. b Mid-esophageal stenosis caused by enlarged lymph nodes in CGDC

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Constipation Prescription

Constipation Prescription

Did you ever think feeling angry and irritable could be a symptom of constipation? A horrible fullness and pressing sharp pains against the bladders can’t help but affect your mood. Sometimes you just want everyone to leave you alone and sleep to escape the pain. It is virtually impossible to be constipated and keep a sunny disposition. Follow the steps in this guide to alleviate constipation and lead a happier healthy life.

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