Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The global epidemic of HIV infection remains appalling. By 2001, there were an estimated 1.4 million HIV-infected children, with 4.5 million deaths. In the UK, paediatric cases are clustered around population centres where there are high concentrations of infected immigrant adults, and to a lesser extent, areas where IV drug abuse is common. The highest incidence remains in London and the southeast. With the national redistribution of immigrant and refugee families, any doctor in any specialty may expect to be involved with children who are HIV positive, or have clinical AIDS. The majority of children are infected vertically, i.e. infection of the infant from an infected mother in the pre-, peri-, or post-natal periods. Rates of transmission vary from 15-20% in the developed countries. Children with HIV infection may have their primary presentation to ENT doctors, who should have appropriate thresholds for suspecting the diagnosis. The most common presenting features include persistent generalised lymphadenopathy, hepatosplenomegaly, chronic/recurrent diarrhoea, poor growth, and fever. Fifteen to twenty percent of untreated children will present with an AIDS-defining illness by 12 months, typically with Pneumocystis pneumonia at approximately 3-4 months of age. Seventy percent of perinatally infected children will exhibit some signs or symptoms by 12 months Without treatment, the median age to progression to AIDS is approximately 6 years, and 25-30% will have died by this age. The median age of death is approximately 9 years. Children may also present with repeated/unusual ear infections, sinus disease (inc. mastoiditis), tonsillitis, orbital/peri-orbital cellulitis, oral candidiasis, and dental infections. Infections with streptococcus pneumoniae and group A streptococcus are common, and often progress to severe systemic infection with an appreciable mortality. Infections may be due to unusual pathogens such as Pseudomonas, 'typical' and atypical Mycobacteria, Candida, Aspergillus, etc. Fungal infections of the sinuses (inc. Aspergillus and Rhizopus spp.) may be particularly devastating, with rapid spread to involve bone and the central nervous system. Another classical presentation, which may present to ENT doctors, is that of bilateral parotid enlargement, especially in children who are 'slow progressors', many of whom also have Lymphoid Interstitial Pneumonitis (LIP). A major attitudinal change has occurred due to advances in 3 main areas: (i) the multidisciplinary management of the infected mother (inc. counselling, antenatal screening, elective caesarean section, advising against breast feeding, etc.), (ii) the prevention of vertical transmission, using anti-retroviral therapy to the infected mother during pregnancy, and to the potentially infected infant in the first weeks of life, and (iii) major advances due to the advent of highly active anti-retroviral treatment. With effective use of these measures, transmission rates may be reduced to <2%. None of the measures though, affect a cure, and it will still be many years before the development of effective vaccines. ENT doctors may be referred children already known to be HIV-positive. Knowing how to talk to infected children (and their parents) is full of potential pitfalls, and requires careful forethought. Many infection-control policies have required considerable rethinking due to the AIDS epidemic. This has especially been the case with respect to needle-stick injuries, post-exposure prophylaxis, sterilization and re-use of equipment, and safe approaches to surgery.
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PMID:HIV infection in children--impact upon ENT doctors. 1466 74

16S rDNA polymerase chain reaction (PCR) in the diagnosis of fastidious organisms is becoming increasingly commonplace. We present the case of a child admitted to an acute paediatric unit of a university teaching hospital with otorrhoea, torticollis, and cervical lymphadenopathy. Examination revealed hepatosplenomegaly associated with pancytopenia. Radiological imaging confirmed a retropharyngeal abscess, bilateral mastoiditis, cerebellar lesions, and venous sinus thrombosis. Swabs of aural discharge grew anaerobes. Drainage of the retropharyngeal abscess and bilateral mastoidectomy were performed. Bone marrow aspiration was initially suspicious of acute leukaemia prompting further investigations, but cytogenetic analysis ruled out this diagnosis and changes were attributed to severe sepsis. Following 27 days of intravenous antibiotics and after clinical improvement, clindamycin was started. Intraoperative pus yielded no significant pathogens. A 16S rDNA PCR confirmed Fusobacterium necrophorum. The boy was discharged on a 6 week course of oral clindamycin.
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PMID:Unusual findings and diagnostic challenges in a child with Lemierre's disease. 2183 43

Pharyngitis is a common clinical complaint for children and accounts for 3.1% of all visits to selected ambulatory care settings. Most children with pharyngitis have benign, self-limited disease with infrequent complications such as peritonsillar abscess, mastoiditis, or lymphadenitis. Recent studies have touted the benefits of steroids in the treatment of children with pharyngitis for pain control. These studies do not address the potential life-threatening complication of steroids in patients with pharyngitis or lymphadenopathy in the setting of undiagnosed acute lymphocytic leukemia (ALL) or lymphoma. We report 4 cases of children treated with steroids for pharyngitis or adenitis that subsequently were diagnosed with ALL or lymphoma. If steroids are to be used in children with pharyngitis or adenitis, the following recommendations should be strongly considered: Careful history and physical examination should be obtained. Presence of hepatosplenomegaly or lymphadenopathy outside the cervical region should raise suspicions regarding an underlying malignancy. Normal results of complete blood cell count in the setting of clear cut pharyngitis with exudates and a lack of significant adenopathy essentially rules out the diagnosis of ALL. Because traditional analgesics are available, which do not affect the curability of ALL or lymphoma, the routine use of steroids in pharyngitis in children should be considered only in rare circumstances.
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PMID:Adverse effects of steroid therapy in children with pharyngitis with unsuspected malignancy. 2286 23