Chronic myelogenous leukemia simulating chronic granulomatous disease.

A 5-year illness of a child, characterized by recurrent bacterial infections and abnormal results of nitroblue tetrazolium dye reduction tests, was suggestive of chronic granulomatous disease but the illness terminated in overt myeloid leukemia. During this progression studies of leukocyte structure and metabolic activity revealed abnormalities that suggested the existence of a "preleukemic" state.     

Chronic granulomatous disease,a heterogeneous syndrome

Chronic granulomatous disease (CGD) is a clinical syndrome, the unifying characteristics of which are a severe predisposition to bacterial and fungal infections, an impaired ability of phagocytic leukocytes to kill certain microorganisms and the failure of these cells to produce microbicidal oxygen metabolites. In CGD the causal biochemical defect and the mechanism of genetic transmission vary from family to family. At least six different molecular defects have been found to underly the X-linked and at least three other the autosomal recessive form of CGD. Diagnosis of carriers is possible in most instances, and prenatal diagnosis by fetoscopic placental vessel puncture has become feasible.     

Chronic granulomatous otitis media in bottle-fed Inuit children.

Otitis media in Inuit children is a problem of relatively recent origin and unknown cause. The prevalence of otitis media in 238 Inuit and 47 Caucasian children in Nain, a small community in Labrador, was determined by examination, and the history of breast-feeding or bottle-feeding was obtained. The prevalence of otitis media was found to be inversely related to the age at which bottle-feeding was started. Clinical observations suggest that otitis media in Inuit children is part of a process leading to chronic foreign body granuloma of the middle ear, and that the granuloma is formed from milk introduced into the relatively short and straight eustachian tubes of Inuit infants by the high negative intraoral pressure necessary for bottle-feeding.     

Chronic granulomatous disease: a review of the infectious and inflammatory complications

Chronic Granulomatous Disease is the most commonly encountered immunodeficiency involving the phagocyte, and is characterized by repeated infections with bacterial and fungal pathogens, as well as the formation of granulomas in tissue. The disease is the result of a disorder of the NADPH oxidase system, culminating in an inability of the phagocyte to generate superoxide, leading to the defective killing of pathogenic organisms. This can lead to infections with Staphylococcus aureus, Psedomonas species, Nocardia species, and fungi (such as Aspergillus species and Candida albicans). Involvement of vital or large organs can contribute to morbidity and/or mortality in the affected patients. Major advances have occurred in the diagnosis and treatment of this disease, with the potential for gene therapy or stem cell transplantation looming on the horizon.     

Chronic granulomatous disease, the McLeod phenotype and the contiguous gene deletion syndrome-a review

Chronic Granulomatous Disease (CGD), a disorder of the NADPH oxidase system, results in phagocyte functional defects and subsequent infections with bacterial and fungal pathogens (such as Aspergillus species and Candida albicans). Deletions and missense, frameshift, or nonsense mutations in the gp91^phox gene (also termed CYBB), located in the Xp21.1 region of the X chromosome, are associated with the most common form of CGD. When larger X-chromosomal deletions occur, including the XK gene deletion, a so-called "Contiguous Gene Deletion Syndrome" may result. The contiguous gene deletion syndrome is known to associate the Kell phenotype/McLeod syndrome with diseases such as X-linked chronic granulomatous disease, Duchenne muscular dystrophy, and X-linked retinitis pigmentosa. These patients are often complicated and management requires special attention to the various facets of the syndrome.     

Chronic obstructive pulmonary disease.

Outpatient management of chronic obstructive pulmonary disease (COPD) is reviewed in this paper. Smoking cessation is probably important, although its benefit in established COPD is unproven. Bronchodilator therapy may be of more than symptomatic benefit and is indicated in virtually all patients. Specific beta 2-agonists are the most widely used agents and can be given in substantially larger doses than are usually recommended. Ipratropium bromide, an anticholinergic drug, is about as effective as a beta 2-agonist, but in large doses the two drugs do not seem to have additive effects, unlike theophylline and beta 2-agonists. Systemic corticosteroids decrease airway obstruction substantially in a small number of patients with COPD; these agents should be reserved for these patients and used sparingly. Inhaled steroids are of little benefit, as are respiratory stimulants and depressants. Broad-spectrum antibiotic therapy helps to relieve symptomatic exacerbations of COPD, particularly those characterized by increased dyspnea, sputum volume and sputum purulence. Cor pulmonale is best managed by diuretics and oxygen, with digoxin reserved for left ventricular failure and supraventricular arrhythmias. Continuous oxygen therapy at home is indicated for the patients who have chronic arterial hypoxemia.     

Quinine-induced granulomatous hepatitis.

Many adverse reactions to quinine have been reported. A 65 year old woman taking quinine sulphate for nocturnal leg cramps presented for investigation of episodes of malaise, fever, nausea, vomiting, and polyarthralgia. Granulomatous hepatitis was diagnosed, for which no common cause was found. She was challenged with quinine sulphate; within hours her temperature had risen and her symptoms returned; transaminase activities rose within 48 hours, as did erythrocyte sedimentation rate. After withdrawal of the drug symptoms abated and transaminase activities returned to normal. The biochemical response to challenge with quinine implicates the drug as the cause of the liver disturbances. Quinine should be added to the list of drugs known to cause granulomatous hepatitis and should be considered in cases where symptoms are episodic or where no other cause is apparent.     

Chronic inflammatory bowel disease in childhood.

The diagnosis of Crohn''s disease in childhood has been facilitated by the use of fibreoptic endoscopy with biopsies, complemented by double-contrast radiology. Clinical suspicion leads initially to several relevant blood tests. These are followed by endoscopy and multiple colonic biopsies or barium follow-through studies depending on whether large-bowel or small-bowel disease is suspected. The present approach to diagnosis is based on corroborative investigative techniques-endoscopy, radiology, and histology, The availability of paediatric colonoscopes of small diameter should make it possible for paediatricians to perform limited examinations, but when more extensive endoscopy is indicated the child should be referred to special centres.