Clinical Improvement After Banding of a Pulmonary Branch Artery in a Symptomatic Patient with Osler–Rendu–Weber Syndrome

We report a symptomatic newborn with Osler–Rendu–Weber syndrome, multiple and diffuse pulmonary arteriovenous malformations, and right-to-left shunting in the left lung. Right-to-left shunting was significantly decreased by selectively banding the left pulmonary branch artery and clipping one large feeding vessel so that total resection of the left lung could be avoided.     

de Quervain thyroiditis in a young boy following hand–foot–mouth disease

de Quervain thyroiditis is a self-limited inflammatory disorder of the thyroid gland. It is an uncommon disease in adults and very rare in children. Fritz de Quervain, a Swiss surgeon, who was an authority on thyroid disease, described the unique pathology of this disease. Granulomatous changes with giant cells in thyroid tissue are the pathological findings. Viral infection in genetically predisposed individuals has been proposed as the pathogenesis of the disease. Clinical hallmarks for the diagnosis are painful thyroid enlargement, elevated erythrocyte sedimentation rate, and C-reactive protein as well as decreased uptake of the thyroid gland on thyroid scintigraphy. In addition, thyrotoxicosis is present in about 50% of cases in early phase of the disease. Serum thyroglobulin level is usually elevated. Only symptomatic treatment with analgesics is usually required for pain relief. Glucocorticoid therapy may be used in severely ill patients. de Quervain thyroiditis is generally completely resolved without complications in 6–12 months. However, permanent hypothyroidism and recurrent disease have been reported in some patients.     

Unusual manifestations of astroblastoma: a radiologic–pathologic analysis

Astroblastoma is a very rare primary glial tumor occurring in children and young adults that is almost exclusively supratentorial in location. We report an extremely unusual presentation of a densely calcified posterior fossa astroblastoma with disseminated spinal and supratentorial metastasis. The mass exhibited neoplastic bone formation, which has not been reported, although calcifications are commonly seen in astroblastomas. A companion case of a low-grade astroblastoma that demonstrated classic histologic features but nonspecific and atypical imaging findings is also included. These cases expand the imaging and pathologic spectrum of this controversial tumor that shows highly variable biologic behavior and is difficult to distinguish from ependymoma.     

Helicobacter Pylori Infection–Related Pancytopenia in a Young Boy

Children with sickle cell anemia (SCA) are faced with complications which may interfere with their educational activities including academic performance. Reports on their academic performance are mainly from developed countries and the results have been inconsistent. This study aimed to determine the academic performance of primary school-aged children with SCA in Nigeria and compare findings with a group of controls. Ninety children with SCA aged 5-11 years were consecutively recruited at the SCA clinic of UNTH Enugu and their age- and sex-matched normal classmates were enrolled as controls. Academic performance of the children with SCA was studied using the overall scores achieved in the three term examinations in the preceding academic year (2009/2010), while their intelligence quotient (IQ) was determined using the Draw-A-Person Test. The findings were compared with that of 90 controls. The mean overall academic score of the children with SCA of 62.71 ± 19.43% was similar to 67.47 ± 16.42% in the controls (P = .077). However, a significantly higher number of children with SCA (32.2% vs. 16.7% of the controls; P = .015) scored below 50%, thus, had poor performance. The mean IQ of the subjects (91.41 ±16.61%) was similar to that of the controls (95.56 ±17.31%, P = .103). However, more SCA patients had lower IQ scores than controls though not statistically significant (P = 0.083). The overall academic performance of children with SCA, therefore, compares favorably with that of controls although there is a higher prevalence of poor performance among them.     

Diagnosis and management of acute pharyngitis in a paediatric population: a cost–effectiveness analysis

Acute pharyngitis is one of the most frequent causes of primary care physician visits; however, there is no agreement about which is the best strategy to diagnose and manage acute pharyngitis in children. The aim of the current study was to evaluate the cost–effectiveness of the recommended strategies to diagnose and manage acute pharyngitis in a paediatric population. A decision tree analysis was performed to compare the following six strategies: “treat all”, “clinical scoring”, “rapid test”, “culture”, “rapid test + culture” and “clinical scoring + rapid test”. The cost data came from the Spanish National Health Service sources. Cost–effectiveness was calculated from the payer’s perspective. Effectiveness was measured as the proportion of patients cured without complications from the disease and did not have any reaction to penicillin therapy; a sensitivity analysis was performed. The findings revealed that the “clinical scoring + rapid test” strategy is the most cost-effective, with a cost–effectiveness ratio of 50.72 €. This strategy dominated all others except “culture”, which was the most effective but also the most costly. The sensitivity analysis showed that “rapid test” became the most cost-effective strategy when the clinical scoring sensitivity was <91% and its specificity was ≤9%. In conclusion, the use of a clinical scoring system to triage the diagnoses and performing a rapid antigen test for those with a high score is the most cost-effective strategy for the diagnosis and management of acute pharyngitis in children. When the clinical scoring system has a low diagnostic accuracy, testing all patients with rapid test becomes the most cost-effective strategy.     

An unusual presentation of May–Thurner Syndrome in a pediatric patient with a pelvic kidney

We report a case of an adolescent who presented with a deep vein thrombosis (DVT) and clinical findings consistent with May–Thurner Syndrome. Specific imaging demonstrated direct compression of the left common iliac vein by an overlying pelvic kidney. The patient's history and clinical presentation is detailed. The discussion focuses on the potential implications for care and management of a patient with an ectopic left pelvic kidney.     

Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997–2003

Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in childhood. In its typical presentation, it is preceded by an episode of diarrhea mostly due to Shiga-toxin-producing Escherichia coli. There is important geographical variation of many aspects of this syndrome. Nationwide data on childhood HUS in Switzerland have not been available so far. In a prospective national study through the Swiss Pediatric Surveillance Unit 114 cases (median age 21 months, 50% boys) were reported between April 1997 and March 2003 by 38 pediatric units (annual incidence 1.42 per 10⁵ children ≤16 years). Shiga-toxin-producing E. coli were isolated in 32 (60%) of tested stool samples, serotype O157:H7 in eight. Sixteen children presented with only minimal renal involvement, including three with underlying urinary tract infection. Six patients presented with atypical hemolytic-uremic syndrome, and six with HUS due to invasive Streptococcus pneumoniae infection. Mortality was 5.3%, including two out of six children with S. pneumoniae infection. The severity of thrombocytopenia and the presence of central nervous system involvement significantly correlated with mortality. In conclusion, childhood HUS is not rare in Switzerland. Contrasting other countries, E. coli O157:H7 play only a minor role in the etiology. Incomplete manifestation is not uncommon.     

In vitro effects of adriamycin: a dose–response study

The in vivo effects of adriamycin (ADR) on the mouse and rat embryos are well described in the literature. However, there is a lack of knowledge about the in vitro effects of ADR. The aim of this study was to investigate the effects of ADR on the developing mouse embryo and to identify a dose of ADR, which could be used for further studies of ADR effects in vitro. CD1 mouse embryos were collected at day 8.5 post conception. They were cultured in the presence of different doses of ADR (0, 125, 250, 375 and 500 μM). After 24 h, the culture was stopped and the embryos (n = 77) were scored morphologically using the Brown–Fabro scoring system and the mean score for each organ was calculated. Dose–response plots were generated and the effective dose 50 (ED50) for each organ was identified from the plots. The effects of ADR on the developing embryo were found to be dose related and there is a dose–response relationship in most of the plots. The dose–response plots were found to be parallel for some organs. A dose of 250 μM ADR was identified as the appropriate dose for further in vitro studies. The effects of ADR on the embryos were dose related and there is a dose-response relationship in most of the developing systems. The presence of parallel dose–response plots for some regions is suggestive of similar mechanism of action of ADR on these regions. A dose of 250 μM of ADR was identified for the first time in the literature and could be used for further studies of the effects of ADR on the mouse foregut in vitro.     

Tetralogy of Fallot in a Patient with Killian–Pallister Syndrome

Killian–Pallister syndrome is a rare dysmorphic condition characterized by specific clinical manifestations and tetrasomy 12p. Although the association of this condition with congenital heart disease has been previously documented, no cases have been reported in association with Fallot's tetralogy. We report one such case.     

Jarcho–Levin Syndrome Associated with a Complex Congenital Heart Anomaly

Jarcho-Levin syndrome is characterized by "crab-like" rib cage deformity and multiple vertebral anomalies that cause respiratory failure. Reports of complex congenital heart defects with this syndrome are rare. We describe a female infant with this syndrome and a complex congenital heart defect and review the literature. Congenital heart defects are occasionally complicated by Jarcho-Levin syndrome. Heart defects involving heterotaxic morphology should be included as one of the important clinical features of this syndrome.     

Focal nodular hyperplasia in a child with Beckwith–Wiedemann syndrome

Beckwith–Wiedemann Syndrome (BWS) is associated with somatic overgrowth and a high risk for embryonal tumors, including hepatoblastoma, a highly malignant liver tumor of childhood. Focal nodular hyperplasia (FNH), on the other hand, is a benign tumor of the liver that is uncommon in childhood. Herein we describe a case of FNH in a child with BWS, the first such report in the literature.     

Slowly Developing Perigraft Seroma After a Modified Blalock–Taussig Shunt

We report a 26-year-old patient with slowly developing perigraft seroma 15 years after a modified Blalock–Taussig shunt with an expanded polytetrafluorethylene graft. The mediastinal mass was first observed on a chest x-ray film 8 years after the shunt operation. The mass contained massive gelatinous or organic tissues with a severely calcified pseudocapsule, and it was adjacent to the calcified but functioning graft. No fluid collections were noted. The histological findings were compatible with a seroma. No recurrence was noted on last follow-up 1 year postoperatively.     

Aortic Calcification in a Patient With Hutchinson–Gilford Progeria Syndrome

We present chest radiographs, echocardiographic image, and selective coronary angiogram of an 18-year old patient with Hutchinson–Gilford progeria syndrome.     

Paclitaxel Drug–Eluting Stent Placement for Pulmonary Vein Stenosis as a Bridge to Heart–Lung Transplantation

Congenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term relief of the stenosis. A case is presented involving single-ventricle physiology associated with extensive and recurrent congenital PVS despite multiple attempts with surgical therapy. Heart–lung transplantation was ultimately performed after drug-eluting stents were placed in pulmonary veins as a bridge to the transplantation.     

Safety, immunogenicity and immediate pain of intramuscular versus subcutaneous administration of a measles–mumps–rubella–varicella vaccine to children aged 11–21 months

This study compared intramuscular and subcutaneous administration of two doses of measles–mumps–rubella–varicella (MMRV) combination vaccine (Priorix-Tetra?, GlaxoSmithKline Biologicals) in children. Healthy children (N?=?328) were randomised to receive MMRV either intramuscularly or subcutaneously. Reactogenicity was similar between treatment groups for immediate vaccination pain, vaccination site pain, redness and incidence of fever and rashes. Slightly less vaccination site swelling occurred during days 0–3 of the post-vaccination period after intramuscular administration. Seroconversion rates for all components, 42–56 days post-dose 2, ranged from 99.3% to 100% in the intramuscular group and from 98.6% to 100% in the subcutaneous. Cell-mediated immunity data supported the humoral immunogenicity findings. In summary, the MMRV vaccine is well tolerated and highly immunogenic when administered either subcutaneously or intramuscularly to children in the second year of life.     

Infant mortality trends in a region of Belarus, 1980–2000

Background

The Chernobyl disaster in 1986 and the breakup of the former Soviet Union (FSU) in 1991 challenged the public health infrastructure in the former Soviet republic of Belarus. Because infant mortality is regarded as a sensitive measure of the overall health of a population, patterns of neonatal and postneonatal deaths were examined within the Mogilev region of Belarus between 1980 and 2000.

Methods

Employing administrative death files, this study utilized a regional cohort design that included all infant deaths occurring among persons residing within the Mogilev oblast of Belarus between 1980 and 2000. Patterns of death and death rates were examined across 3 intervals: 1980–1985 (pre-Chernobyl), 1986–1991 (post-Chernobyl &; pre-FSU breakup), and 1992–2000 (post-Chernobyl &; post-FSU breakup).

Results

Annual infant mortality rates declined during the 1980s, increased during the early 1990s, and have remained stable thereafter. While infant mortality rates in Mogilev have decreased since the period 1980–1985 among both males and females, this decrement appears due to decreases in postneonatal mortality. Rates of postneonatal mortality in Mogilev have decreased since the period 1980–1985 among both males and females. Analyses of trends for infant mortality and neonatal mortality demonstrated continuous decreases between 1990, followed by a bell-shaped excess in the 1990's. Compared to rates of infant mortality for other countries, rates in the Mogilev region are generally higher than rates for the United States, but lower than rates in Russia. During the 1990s, rates for both neonatal and postneonatal mortality in Mogilev were two times the comparable rates for East and West Germany.

Conclusions

While neonatal mortality rates in Mogilev have remained stable, rates for postneonatal mortality have decreased among both males and females during the period examined. Infant mortality rates in the Mogilev region of Belarus remain elevated compared to rates for other western countries, but lower than rates in Russia. The public health infrastructure might attempt to assure that prenatal, maternal, and postnatal care is maximized.