Antinuclear antibody status and risk of death in children and adolescents.

OBJECTIVES: The association between the presence of antinuclear antibodies (ANA) and mortality has been rarely reported. The present study explored the value of ANA as a predictor of overall survival in children and adolescents. METHODS: Patients younger than 20 years who underwent ANA testing in Chang Gung Memorial Hospital (CGMH) from 2000 to 2008 were enrolled in this study. Mortality was ascertained by using the National Death Registry of Taiwan. Positive ANA titres were categorized as low (1:40 to 1:80), medium (1:160 to 1:320), and high (≥ 1:640). RESULTS: A total of 13 345 subjects (6579 males, 6766 females) were enrolled during the 9-year study period. The overall prevalence of low, medium, and high ANA titres was 20.8% (n = 2774), 6.0% (n = 804), and 2.5% (n = 338), respectively. During 45,140 person-years of follow-up, 146 deaths were identified and the crude mortality rates were 3.8 and 3.0 per 1000 person-years for subjects with positive and negative ANA test results, respectively (p = 0.130). Compared with ANA-negative subjects, the adjusted hazard ratio (HR) for all-cause mortality among those with a high ANA titre was 5.18 [95% confidence interval (CI) 3.13-8.57]. A low-to-medium ANA titre was not associated with increased mortality. Among the 18 deaths in individuals with a high ANA titre, 14 were due to systemic lupus erythematosus (SLE). In comparison, five out of 34 deaths among those with low-to-medium titres of ANA and none of those with negative ANA were related to SLE. CONCLUSIONS: Children and adolescents with high ANA titres should receive greater attention and monitoring to prevent unfavourable outcomes because they have a higher mortality risk than those with negative ANA results.

Complicated features in a young child with influenza B virus pneumonia and co-infection with Stenotrophomonas maltophilia.

A 3.5-year-old child with influenza B virus pneumonia developed pneumomediastinum and subcutaneous emphysema on the 3rd day of illness. Bronchoscopy demonstrated obstruction of the left main bronchus by mucopurulent sputum. Culture of the broncho-alveolar lavage yielded Stenotrophomonas maltophilia. After the respiratory complications resolved (11 days), the patient developed neurological symptoms and was diagnosed as acute disseminated encephalomyelitis (ADEM). Stenotrophomonas maltophilia was probably a factor in the development of pneumomediastinum. To our knowledge, this is the first case report of influenza virus infection with Stenotrophomonas maltophilia co-infection associated with spontaneous pneumomediastinum.

Influenza B-associated rhabdomyolysis in Taiwanese children.

AIM: Influenza B-associated rhabdomyolysis (IBAR) is an infrequent and little-known complication of influenza B virus infection in children. Diagnosis is usually made based on clinical history, the presence of influenza in the community and detection of virus in nasopharyngeal specimens. The aim of this study was to describe the clinical and laboratory manifestations, complications and outcomes of IBAR in Taiwanese children. METHODS: A retrospective analysis was conducted in patients aged < 17 years who had been diagnosed with IBAR at a university children's hospital in North Taiwan during 2000-2007. All children enrolled in the study had presented with rhabdomyolysis associated with laboratory-confirmed influenza B infections. Demographic data, clinical manifestations, complications and outcomes were included in the analysis. RESULTS: Overall, 24 IBAR cases were analysed. IBAR typically occurred in school-aged children with a 7:3 male:female ratio. The mean age was 7.2 ± 1.9 years. Nearly 63% of cases occurred between the ages of 6 and 9 years. The median interval between the onset of influenza and onset of IBAR was 3.4 days (range, 1-14). The calf muscles were involved in all cases. Laboratory tests indicated a mean initial blood creatine kinase of 4212 U/L. The median time to clinical recovery was 5 days (range 1-24). No patient had renal failure. IBAR tends to occur mainly in winter and spring during influenza B outbreaks. IBAR sometimes induces some complications, and early detection and careful medical treatment are necessary. CONCLUSION: The results of this study indicate that outcomes of IBAR are good with proper medical care.

Clinical and radiographic spectrum of septic pulmonary embolism.

AIMS: To review the clinical presentation, radiographic findings, and outcome of therapy in children with septic pulmonary embolism. METHODS: Retrospective analysis of patients in a tertiary paediatric facility in northern Taiwan. RESULTS: Ten children were identified with septic pulmonary emboli in a four year retrospective chart review between 1998 and 2001. Seven were immunocompetent, two were premature infants, one had beta thalassemia major. Seven had community acquired staphylococcal infections and bacteraemia, of which six were methicillin resistant Staphylococus aureus (MRSA) isolates. Five had soft tissue infections, two bone infections, one suppurative otitis media, one catheter related infection, and one unknown foci of infection. Multiple and bilateral nodular pulmonary parenchymal lesions were common on plain chest radiographs, but chest computed tomography scans showed the additional findings of a "vessel sign" and central cavitations, confirming the existence of septic pulmonary embolism. CONCLUSIONS: Community acquired MRSA infections occurred in seven patients with septic pulmonary embolism but without predisposing high risk factors. Critically ill children with skin, soft tissue, or bone infections, when associated with septic pulmonary embolism in an area with a high rate of MRSA, should be empirically treated with glycopeptides (such as vancomycin or teicoplanin) before susceptibility results are known, in order to minimise morbidity and avoid mortality.

Modern pharmacotherapies for type 2 diabetes mellitus.

UNLABELLED: CME CreditsThe National Medical Association (NMA) is accredited by the Accreditation Council for Continuing Medical Education to sponsor continuing medical education (CME) for physicians.Readers:NMA certifies that when CME material is used as directed it meets the criteria for 2 hours of credit in Category I toward the NMA Physician Achievement Award (PAA) or American Medical Association Physician's Recognition Award (PRA).Each physician should claim only those hours of credit that the physician actually spent in the educational activity. Estimated time for reading the text and taking the CME quiz is approximately 2 hours. The NMA will not maintain any records on individual performance.Physician readers will receive 2 category I credits upon correctly answering a minimum of 70% of the CME questions at the end of this CME article. Physician readers must send the answers to JNMA/CME, 1012 Tenth St., NW, Washington, DC 20001. Answers to the CME questions accompanying this article must be marked by September 25, 2001.Authors:The first or second author of articles published in the Journal of the National Medical Association may claim Category I credit for the NMA PAA or the American Medical Association PRA by including a reprint of the article's first page as part of the PRA or PAA application. APPLICATIONS: PAA application can be obtained by calling NMA's CME office at (202) 347-1895, ext. 241. PRA application can be found by visiting http://www.ama-assn.org/med-sci/pra/prahdr.htm.

Idiopathic pulmonary hemosiderosis in a child: report of one case.

Idiopathic pulmonary hemosiderosis (IPH) is uncommon in children. We report a 3-year-old girl who was presented with acute pale-looking appearance, hemoptysis, hematemesis and shortness of breath. This patient was confirmed to have pulmonary hemorrhage by the presence of hemosiderin-laden macrophages in the bronchoalveolar lavage fluid using a flexible bronchoscope. Other causes of PH including glomerular, cardiac and immunological disorder were excluded by normal laboratory studies. She was primarily treated by oral prednisolone, but due to recurrent hemoptysis, immunosuppressive agent was added for maintenance therapy. Pediatricians should consider PH in a patient who has recurrent dyspnea, hemoptysis and iron deficiency anemia.

A family cluster of streptococcal toxic shock syndrome in children: clinical implication and epidemiological investigation.

BACKGROUND: Most invasive group A streptococcal (GAS) disease occurs sporadically. Reports of family clusters of these infections are scanty, and most invasive disease occurs in adults. We describe a family cluster of streptococcal toxic shock syndrome (STSS) involving 3 children and present the results of an epidemiologic investigation. PATIENTS AND METHODS: During a 16-day period, 3 children in a family developed STSS with an interval of 7 and 9 days, respectively, between the onset of disease. Cases 2 and 3 had GAS isolated from blood culture. Case 2 was fatal. Pharyngeal culture survey of the family members and schoolchildren was conducted. Antibiogram, serotyping, detection of exotoxin genes, and random amplified polymorphic DNA patterns of the disease strains and survey strains were examined. RESULTS: One of 15 family members sampled-the sister of the index case-and 7 (5.6%) of 125 schoolchildren sampled had GAS isolated from pharyngeal cultures. Of the 10 strains examined, 2 isolates from the patients, 1 from the sister of index case, and 2 from the classmates of case 2 (the fatal case) had an identical pattern of both genotype and phenotype. CONCLUSION: We describe a family cluster of STSS involving 3 children caused by a single clone and provide additional data regarding invasive GAS infection subsequent to household contact. Additional studies should be conducted in conjunction with surveillance to define better the magnitude of risk in household contacts and to identify settings in which subsequent infections may occur.

Tumor lysis syndrome in an infant with Langerhans cell histiocytosis successfully treated using continuous arteriovenous hemofiltration.

Langerhans cell histiocytosis (LCH) is an enigmatic disease usually occurring in children. Tumor lysis syndrome (TLS) is a clinical syndrome associated with severe metabolic derangement and oliguric acute renal failure. In this report, we present the clinical course of an infant with advanced LCH who had TLS develop after chemotherapy. Treatment with continuous arteriovenous hemofiltration resulted in effective control of serum uric acid, potassium, creatinine, phosphorus, and blood urea nitrogen levels in the blood.

Genetic diagnosis of familial hypercholesterolemia in affected relatives using pedigree tracing.

OBJECTIVES: To describe a process of diagnosing familial hypercholesterolemia (FH) at the DNA level in selected family members of affected individuals. DESIGN AND METHODS: A 63-year-old male patient presented with cholesterol elevations consistent with heterozygous familial hypercholesterolemia. Through participation with the international "MEDPED FH" project to detect affected relatives and to identify their LDL-receptor mutation, the patient was discovered to carry the Lebanese mutation, whereby the codon for cysteine at residue 660 instead codes for a premature termination (C660X), thus truncating the protein product. This mutation also created a new restriction recognition site for the endonuclease Hinfl, which permitted rapid detection of the mutation in selected family members using restriction fragment-length polymorphisms. RESULTS: The patient's son, who had cholesterol levels consistent with heterozygous FH, was also found to be a heterozygote for the C660X variant of the LDL-receptor. CONCLUSIONS: Diagnosis of familial hypercholesterolemia at the DNA level is possible as a relatively rapid screening technique in families with a known LDL-receptor mutation, established through participation with the MED-PED FH project.

Successful outcome in severe pregnancy-associated hyperlipemia: a case report and literature review.

Severe hypertriglyceridemia causing pancreatitis is a rare complication of pregnancy, usually occurring in the second and third trimesters. Treatment includes a very low-fat diet, intravenous fluids, total parenteral nutrition, and plasma apheresis. In this article, the authors report the case of a pregnant woman who presented with a plasma triglyceride level of 65 mmol/L, abdominal pain, and a threatened abortion at 8 weeks of gestation. Treatment included restriction of dietary fat to below 10% of total calories, liquid protein supplementation, multiple hospitalizations for treatment with intravenous fluids, and total parenteral nutrition. Continuous intravenous heparin was started at 29 weeks of gestation for pulmonary embolism. This was associated with a dramatic decrease in plasma triglyceride levels. A normal female child was born at 37 weeks of gestation. The mother's weight at 2 weeks postpartum was 15 lb below her pregnant weight. It was concluded that a successful pregnancy is possible even when plasma triglyceride levels are very high early in the pregnancy.

Restriction isotyping of apolipoprotein E R145C in type III hyperlipoproteinemia.

BACKGROUND: An adolescent female who presented with type III hyperlipoproteinemia was found to have an E3/2 phenotype by isoelectric focusing while restriction isotyping using HhaI revealed a pattern compatible with classical E3/3. Studies were carried out to determine the nature of the patient's apolipoprotein E abnormality. METHODS: A 244 bp fragment of exon 4 of apolipoprotein E was amplified by the polymerase chain reaction (PCR). Restriction isotyping was carried out with BbvI and Fnu4HI and results were confirmed by direct sequencing. RESULTS: The patient was found to be heterozygous for a C-->T transition at the first base of codon 145, resulting in a substitution of cysteine for arginine (R145C) which completely explained the discrepancy between the isoelectric focusing and HhaI restriction isotyping. We noted that the DNA change altered palindromic recognition sites for the endonucleases BbvI and Fnu4HI. CONCLUSIONS: Digestion with BbvI or Fnu4HI allows for rapid restriction isotyping for the rare apolipoprotein E R145C mutation associated with hyperlipidemia.

Ascariasis associated with acute pancreatitis in a child.

A six-year-old aboriginal girl living in northeast Taiwan, was admitted via Emergency Service with the chief complaint of epigastralgia for one day. Fever, leucocytosis, hyponatremia, acidosis and unilateral pleural effusion were noted. The serum amylase was 2976 U/L. Image studies including abdominal sonography and computerized tomography revealed a swollen and edematous pancreas. There was no evidence of previous trauma, drug using, hereditary problems, nor biliary tract stone; the patient was noted to have adult worms and eggs of Ascaris lumbricoides in stool and vomitus. Ascariasis associated with pancreatitis was suspected. She recovered completely after antihelmintic therapy (mebendazole) and supportive treatment. Fourteen ascarides and 20 trichurides were expelled during nine days of admission.