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Vol 7, No 2
Vol 7, No 2 May 2011 ISSN 1708-8569
Review articles
Original articles
Original article/CME
Brief report
Clinical practice
Case reports
Working conditions and female reproductive health
  Dag Bratlid
  [Abstract] [Full Text] [PDF]  
Review articles:
The puzzling clinical spectrum and course of juvenile sarcoidosis
  Andrew Fretzayas, Maria Moustaki, Olga Vougiouka

Author Affiliations: Third Department of Pediatrics, University of Athens, School of Medicine "Attikon" University Hospital, Athens, Greece (Fretzayas A, Moustaki M); Second Department of Pediatrics, University of Athens, School of Medicine, "P & A Kyriakou" Children's Hospital, Athens, Greece (Vougiouka O)

Corresponding Author: Andrew Fretzayas, MD, PhD, Third Department of Pediatrics, "Attikon" University Hospital, Athens University School of Medicine,1 Rimini str, Haidari, 12462 Athens, Greece (Tel: 00302105831299; Fax: 00302105832229; Email: mar.moustaki@gmail.com)

doi: 10.1007/s12519-011-0261-0

Background: Juvenile sarcoidosis is a rare, chronic, multisystem, granulomatous disease of obscure etiology which is seen in childhood and adulthood. The disease in childhood has a course different from that in adulthood.

Data sources: PubMed database was searched using terms sarcoidosis, children or childhood sarcoidosis or juvenile sarcoidosis in combination with one of the following terms: epidemiology, clinical manifestations, genetics, diagnosis, treatment, and prognosis. We also retrieved the terms such as early onset sarcoidosis and Blau syndrome. Furthermore, e-medicine and European Respiratory Society monographs for sarcoidosis were reviewed.

Results: Sarcoidosis in childhood presents with two age dependent, distinct forms. In younger children it is clinically evident before the age of four years and characterized by the triad of rash, arthritis and uveitis. In their older counterparts, the juvenile late onset sarcoidosis involves several organs and its clinical appearance resembles the adult type of the disease, with the respiratory system being most frequently affected (hilar lymphadenopathy, pulmonary infiltrations). Steroid is the main agent of treatment whereas methotrexate is also used for beneficial steroid sparing effects. New, novel therapies may change the outcome of the disease especially in difficult morbid cases.

Conclusions: Sarcoidosis in childhood is recognized as a systemic disease affecting various organs and having diverse clinical course depending on the age of onset.

Key words: corticosteroids; early onset sarcoidosis; juvenile sarcoidosis; late onset sarcoidosis; methotrexate

World J Pediatr 2011;7(2):103-110

  [Abstract] [Full Text] [PDF]  
Congenital atrichia and hypotrichosis
  Antoni Bennàssar, Juan Ferrando, Ramon Grimalt

Author Affiliations: Department of Dermatology, Hospital Clínic, University of Barcelona, Barcelona, Spain (Bennàssar A, Ferrando J, Grimalt R)

Corresponding Author: Ramon Grimalt, MD, Department of Dermatology, Hospital Clínic, Villarroel 170, 08036 Barcelona, Spain (Tel: + 34 93 2275400 (Ext 2422); Fax: + 34 93 2275438; Email: ramongrimalt@gmail.com)

doi: 10.1007/s12519-011-0262-z

Background: Alopecia present from birth includes a broad differential diagnosis and often represents a diagnostic and therapeutic challenge for the involved physician.

Data sources: An initial correct diagnosis and classification is essential because structural hair defects may be the expression of a genetic disorder affecting hair growth, part of a congenital syndrome with accompanying hair malformations, or a marker for an underlying metabolic disorder and may impact the mental and physical development of a child. Pathological hair loss rarely occurs in the first year of life; however, it may be a leading symptom of many congenital diseases.

Results: In recent years, the clinical and microscopic features of hereditary hair shaft disorders have been characterized and classified. Furthermore, significant progress has been made in our knowledge of genes that control the normal development and differentiation of hair follicles, and thus the research is to define and classify the hair disorders within a genetic basis.

Conclusions: In this article we discuss several types of genotrichosis and provide a practical classification based on their clinical features.

Key words: alopecia; disorders; gentrichosis; hair growth; hypotrichosis

                   World J Pediatr 2011;7(2):111-117

  [Abstract] [Full Text] [PDF]  
Chest-deformities: a proposal for a classification
  G.H. Willital, A.K. Saxena, U. Schütze, W. Richter

Author Affiliations: Pediatric Surgical Clinic, University Clinic Graz, Austria (Saxena A); Pediatric Surgical Research Institute Münster, Germany (Schütze U); Surgical Clinic Hospital Mühldorf/Inn, Munich, Germany (Richter W)

Corresponding Author: Günter H. Willital, Professor, MD, Pediatric Surgical Research Institute Münster, Am Getterbach 49e, D-48163 Münster, Germany (Tel: +49-251-717555; Fax: +49-251-712661; Email: g.willital@web.de)

doi: 10.1007/s12519-011-0263-y

Background: In this article we assess the significance of classifying chest-deformities based on morphological findings in type-related treatment and its results.

Data sources: Recent publications on chest-deformities in children and youth were retrieved from PubMed and Medline and from our clinical and intraoperative findings.

Results: Chest-deformities are diagnosed by thorax-measurements using a flexible meter projected on a graph-paper by MR/CT investigations and color coded videorasterstereography. In addition an ultrasound guided mediastinal analysis is performed on the heart, the great vessels and mediastinal organs. These investigations could determine meticulously the morphology of the sternum, the sterno-costal segments and the costal arch, enabling to find different chest wall deformities, i.e., 11 different types. The clinical and surgical significance of such a classification can be shown by comparing postoperative results of non-classified chest-deformities with those of classified. Preoperatively non-classified chest-deformities often have postoperative asymmetric shapes, partial local recurrences, costal arch eversions and a platythorax. Such a classification can be used to analyze and predict so-called "secondary associated alterations" of the vertebral column or mediastinal organs.

Conclusions: Determining the specific type of a thorax deformity could be considered a type-related physiotherapy as conservative treatment or vacuum treatment and if surgery is indicated a type-related surgical correction can be performed. A type-related and adapted surgical correction can prevent subsequent mitral valve prolapse, recurrent infections, vertebral disturbances caused by kypho-scoliosis and increasing psychological irritation. Typing chest-deformities are an additional and essential help for the surgeon to perform specific surgical procedures: detorsion of the sternum, correction of the sterno-costal region, the costal arch bow and the kind of chest wall immobilization by metal struts. It can also compare the postoperative results more accurately in similar types of chest-deformities.

Key words: chest-deformities; classification; funnel chest

                    World J Pediatr 2011;7(2):118-123

  [Abstract] [Full Text] [PDF]  
Airway remodeling: a potential therapeutic target in asthma
  Wei-Xi Zhang, Chang-Chong Li

Author Affiliations: Department of Respiratory Medicine, Yuying Children's Hospital, Wenzhou Medical College, Wenzhou, China (Zhang WX, Li CC)

Corresponding Author: Chang-Chong Li, Department of Respiratory Medicine, Yuying Children's Hospital, Wenzhou Medical College, Wenzhou, China (Tel: 86-577-88816281; Fax: 86-577-88832693; Email: wzlichch@21cn.com)

doi: 10.1007/s12519-011-0264-x

Background: Apart from airway inflammation, airway remodeling is one of the main pathological features of asthma. However, it remains unclear when airway remodeling starts in children and whether it could be a potential therapeutic target in asthma.

Data sources: We have reviewed the recent literature regarding structural changes after airway remodeling, the relationship between airway inflammation and airway remodeling, the relationship between childhood asthma and airway remodeling, and the role of long-term medication in asthma treatment for airway remodeling.

Results: The relationship between airway inflammation and airway remodeling is still controversial. A number of morphological and pathological studies have confirmed that airway remodeling occurs not only in adult asthma, but also in childhood asthma. It develops early in the disease process of asthma. At present, long-term medication in asthma treatment mainly focuses on anti-inflammation. However, there are no therapeutic interventions that revert airway remodeling once it is established.

Conclusions: Airway remodeling may provide a possible new therapeutic target in the management of asthma. It is imperative to strengthen the research in developing new medications specifically for asthma airway remodeling. Prevention and treatment of airway remodeling become top priority in future asthma research.

Key words: airway remodeling; asthma; children; inflammation


World J Pediatr 2011;7(2):124-128

  [Abstract] [Full Text] [PDF]  
Original articles:
Effect of rotating shift work on childbearing and birth weight: a study of women working in a semiconductor manufacturing factory
  Yu-Cheng Lin, Mei-Huei Chen, Chia-Jung Hsieh, Pau-Chung Chen

Background: Stable circadian rhythm is important for both maternal and fetal health. This retrospective analysis of women in a semiconductor factory evaluated the effect of shift work exposure on childbearing and birth weight.

Methods: Records of 440 female employees (initial mean age: 28.4 years) including 111 mothers who had 158 live births during the period of observation (1997-2007) were reviewed. The data analyzed included maternal age, general health condition, highest educational level, life-style and occupational factors, as well as newborn gender, birth weight, birth order and gestational age.

Results: The childbearing rates of female workers on three different work schedules (consistent daytime work (CDW), intermittent (i-) or persistent (p-) rotating shift works (RSW)) were 32.1%, 20.0% and 25.4%, respectively (P=0.047). After controlling for potential confounding factors, childbearing rates among women with CDW exceeded those of shift workers (odds ratio (OR), 1.7; 95% confidence interval (CI), 1.0-3.0). The birth weights of newborns from mothers on the three work schedules (CDW, i-RSW and p-RSW) were significantly different (3271.7±395.4, 3251.3±460.9, and 2998.5±381.2 g, respectively (P<0.01). Newborns within the lightest birth weight quintile were significantly more likely to be born to mothers with exposure to p-RSW (OR, 4.3; 95% CI, 1.1-16.8).

Conclusions: Rotating shift work exposure was significantly associated with decreased childbearing and lighter birth weight in women working in this semiconductor manufacturing factory. Work schedules should be carefully planned for female employees who are pregnant or preparing for pregnancy. Prenatal evaluations for mothers with persistent day-night rotating shift work exposures are especially necessary.

Key words: birth weight; childbearing; rotating shift work

World J Pediatr 2011;7(2):129-135

  [Abstract] [Full Text] [PDF]  
Ethnicity differences in plasma apoC-III levels between African American and Caucasian youths
  Ji-Young Lee, Hye-Ryun Hong, Hyun-Sik Kang

Author Affiliations: Division of Humanities and Social Sciences, Pohang University of Science and Technology, Pohang, Republic of Korea (Lee JY); School of Sports Science, Sungkyunkwan University, Suwon, Republic of Korea (Hong HR, Kang HS)

Corresponding Author: Hyun-Sik Kang, PhD, School of Sports Science, Sungkyunkwan University, 300 Chenchen-dong, Jangan-gu, Suwon, Republic of Korea (Tel: +82-31-299-6911; Fax: +82-31-299-6929; Email: hkang@skku.edu)

doi: 10.1007/s12519-011-0266-8

Background: Little is known about the association between apoC-III and lipoprotein-lipids in African American (AA) and Caucasian (CA) youths. The aim of this study was to investigate if plasma apoC-III level is associated with ethnicity differences in atherogenic lipoprotein-lipids between AA and CA youths.

Methods: A total of 202 youths (mean age 16.1±1.3 y, range 13.8-18.9 y) consisting of 122 AA (boys/girls, 52/70) and 80 CA (boys/girls, 40/40) youths were recruited via flyers sent to local high schools. For AA youths, body mass index (BMI) values were 22.5±5.0 kg/m2 and 25.0±6.8 kg/m2 for boys and girls, respectively. For CA youths, BMI values were 22.0±4.8 kg/m2 and 22.1±5.0 kg/m2 for boys and girls, respectively. Anthropometric variables were measured using standard procedures. Body fat was measured by dual-energy X-ray absorptiometry. Fasting glucose and insulin, lipoprotein-lipids, and apolipoproteins were measured in fasting plasma samples.

Results: AA youths had significantly lower values in apoC-III (P<0.001), triglyceride (P<0.001), and total cholesterol/high-density lipoprotein cholesterol (P=0.011) and higher values in HDLC (P=0.004), apoE (P=0.016), insulin (P=0.027), and homoeostasis model of assessment-insulin resistance (HOMA-IR) (P=0.025) than CA youths. Body composition and insulin resistance parameters were significantly associated with apoC-III levels in CA youths, but not in AA youths. Regression analyses showed that waist circumference and HOMA-IR were significant predictors for apoC-III in CA, not AA, youths.

Conclusions: The findings of the current study suggest that ethnicity differences in atherogenic lipids between AA and CA youths may be associated with differences in apoC-III and apoE levels.

Key words: adiposity; ethnicity; lipoprotein-lipids; pediatrics; risk factors

                    World J Pediatr 2011;7(2):136-142

  [Abstract] [Full Text] [PDF]  
Poison exposure and outcome of children admitted to a pediatric emergency department
  Yan-Ren Lin, Tung-Kung Wu, Tzu-An Liu, Chu-Chung Chou, Han-Ping Wu

Author Affiliations: Department of Emergency Medicine, Changhua Christian Hospital, Changhua, Taiwan, China (Lin YR, Chou CC); Department of Pediatrics, Buddhist Tzu Chi General Hospital, Taichung Branch, Taichung, Taiwan, China (Wu HP); Department of Biological Science and Technology and Institute of Biochemical Engineering, National Chiao Tung University, Hsinchu, Taiwan, China (Lin YR, Wu TK); Institute of Cellular and System Medicine, National Health Research Institutes, Zhunan, Miaoli County, Taiwan, China (Liu TA); Institute of Medicine, Chungshan Medical University, Taichung, Taiwan, China (Chou CC); Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan (Wu HP); Department of Medicine, Tzu Chi University, Hualien, Taiwan, China (Wu HP)


Corresponding Author: Dr. Han-Ping Wu, Department of Pediatrics, Buddhist Tzu Chi General Hospital, Taichung Branch, Taichung, No.66, Sec. 1, Fongsing Rd., Tanzih Township, Taichung County 42743, Taiwan, China (Tel: 886-4-36060666; Fax: 886-4-36021123; Email: arthur1226@gmail.com)

doi: 10.1007/s12519-011-0267-7

Background: This paper reports the characteristics, outcomes and clinical features of children with poisoning treated at an emergency department (ED).

Methods: This retrospective study at an emergency department consisted of 140 children with poison exposure who were aged under 18 years. Their characteristics were analyzed in order to understand the differences between accidental and non-accidental poisoning. The poisonous materials were divided into two major categories (pharmaceuticals and non-pharmaceuticals) and their associations with patient outcomes were analyzed. Furthermore, the association was analyzed between the incidence of poison exposure and the season in which the poison exposure occurred.

Results: The incidence of poison exposure was highest among adolescents and pre-school age children. Non-accidental poisoning was more common in older girls and accidental poisoning was more common in younger boys (P<0.001). Neurological system agents were the most common cause of poisoning in the pharmaceutical group and cleansing products were the most common cause of poisoning in the non-pharmaceutical group. Neurological and gastrointestinal symptoms were the most common clinical presentations for the pharmaceutical and non-pharmaceutical groups, respectively. Furthermore, poisoning due to cleansing products and analgesics were associated with the longest duration of hospitalization. March was the highest risk month for pediatric poisoning (P=0.018).

Conclusions: Cleansing products and analgesics were associated with the longest duration of hospitalization and intentional poison was more common in girls.

Key words: children; cleansing products; duration of hospitalization; non-pharmaceuticals;  pharmaceuticals; poison  

World J Pediatr 2011;7(2):143-149

  [Abstract] [Full Text] [PDF]  
Synergistic effects of iron deficiency and lead exposure on blood lead levels in children
  Dilshad Ahmed Khan, Wafa Munir Ansari, Farooq Ahmad Khan

Author Affiliations: Department of Pathology, Army Medical College, National University of Science and Technology, Islamabad, Pakistan (Khan DA,  Ansari WM, Khan FA)

Corresponding Author: Khan DA, Army Medical College, University of Science & Technology, Islamabad, Pakistan (Tel: 92519273649; Email: dilshad@nust.edu.pk; dilshad56@yahoo.com)

doi: 10.1007/s12519-011-0257-9

Background: Lead poisoning is a well recognized environmental health problem in children. Independent association of iron deficiency and lead exposure with elevated blood lead level (BLL) has been reported. Whether iron deficiency in combination with chronic lead exposure increases BLL and susceptibility to its harmful effects in children needs to be elucidated.

Methods: In this case-control study, 246 children were randomly recruited. They comprised 123 children of lead smelters/battery recycle plant workers living close to the industries at Wah/Gujranwala, Pakistan (lead exposed group) and 123 children living 30 km away from the industrial area (controls). Blood lead analysis was carried out on the anodic stripping voltammeter lead analyzer 3010B. Blood counting was done on a Sysmex hematological analyzer and serum ferritin was determined by kit method on Immulite-1000.

Results: Of the 123 children in each group, 42 (34%) were iron deficient in the exposed group while 35 (28%) in the controls. The children's median age was 4 years (69 males and 54 females in each group). Lead exposed iron deficient children had significantly higher BLL median (quartile) 13.1 μg/dL (10.1-16.8) as compared with 9.6 μg/dL (7.6-10.3) in the iron deficient controls (P<0.05). Elevated BLL level was found in 31% of the lead exposed children and in 11% of the controls. Lead exposed children revealed a stronger negative correlation (r= -0.54; P=0.001) between BLL and serum ferritin than the controls (r=-0.36; P=0.01).

Conclusion: Iron deficiency in combination with lead exposure synergistically elevates blood lead levels and susceptibility to its harmful effects in children.

Key words: blood lead levels; children; iron deficiency; lead exposure; serum ferritin

                   World J Pediatr 2011;7(2):150-154

  [Abstract] [Full Text] [PDF]  
Swaddling: a traditional care method rediscovered
  Lars Eckehard Meyer, Thomas Erler

Author Affiliations: Carl-Thiem-Klinikum Cottbus gGmbH, Clinic for Paediatric and Adolescent Medicine, Academic Teaching Hospital of the Charité – Universitätsmedizin Berlin, Cottbus, Germany (Meyer LE, Erler T)

Corresponding Author: Lars E Meyer, Carl-Thiem-Klinikum Cottbus, Klinik für Kinder- und Jugendmedizin, Thiemstr. 111, 03048 Cottbus, Germany (Tel: +49 355 46 2336; Fax: +49 355 46 2077; Email: l.meyer@ctk.de)

doi: 10.1007/s12519-011-0268-6

Background: This study was undertaken to compare the sleep profiles of healthy infants in swaddling and sleeping bag conditions.

Methods: Polysomnographs of 85 healthy infants (40 in the study group, 45 in the control group) with a mean age of 7.5 weeks were recorded in the sleeping laboratory. A positive decision from the local Ethics Committee and the written consent of the parents were obtained for the study.

Results: Swaddling significantly reduces the rate of spontaneous waking (events/h: 1.39 [0.85-2.77] vs. 2.81 [1.49-4.53], P=0.020) and the number of sleep stage changes (events/h: 3.82 [2.97-5.16] vs. 5.37 [3.58-6.67], P=0.015). Swaddling promotes quiet sleep (36.37% [29%-40.31%] vs. 30.2% [24.45%-36.78%], P=0.032), the time spent awake was decreased (8.98% [4.62%-14.25%] vs. 14.17% [9.2%-18.94%], P=0.001) and sleep efficiency was increased (91.02% [85.75%-95.38%] vs. 85.83% [81.06%-90.8%], P=0.001).

Conclusion: Swaddling promotes a more quiet sleep in infants.

Key words: infantile regulatory disorder; polysomnography; sudden infant death syndrome; swaddling

                   World J Pediatr 2011;7(2):155-160

  [Abstract] [Full Text] [PDF]  
Original article/CME:
Comparison of methods for teaching developmental milestones to pediatric residents
  M Leiner, G Prasad Krishnamurthy, O Blanc, B Castillo, I Medina

Author Affiliations: Department of Psychiatry and Center of Excellence for Neurosciences, Texas tech University Health Sciences Center, El Paso, Texas, USA (Leiner, M); Department of Pediatrics, Texas Tech University Health Sciences Center, El Paso, Texas, USA (Krishnamurthy GP, Blanc O, Castillo B, Medina I)

Corresponding Author: Marie Leiner, Ph.D., 4800 Alberta, El Paso, Texas, 79905, USA (Tel: 915 545-7590 Fax: 915 545-7571; Email: marie.leiner@ttuhsc.edu)

doi: 10.1007/s12519-011-0269-5

Background:  Interactive media are effective tools in teaching and allow for self-directed study, which develops skills for life-long learning among health professionals. With this type of study, the learner can review material at his/her own pace and target areas that require emphasis. Pediatric residents require an accurate understanding of developmental milestones in children of various ages and their normal variations. The best representation of developmental milestones requires video recordings of children demonstrating appropriate skills. However, such recordings are not always available, so printed materials are most often used. In this realm, a computer-based interactive learning tool using animated cartoons gives flexibility for presentation using drawings. We compared pediatric residents' knowledge of developmental milestones in neonates to 5-year-olds before and after study with either an interactive DVD or paper-based materials.

Methods: A team of physicians and educational specialists in Texas Tech University Health Sciences Center produced an interactive DVD containing animated cartoons and questions. Residents were divided into an intervention group that used the DVD and a control group that used traditional paper-based materials. Each group's improvement [or increase] in knowledge was measured.

Results: Compared with the 17 residents in the control group, the 37 in the intervention group tended to acquire more knowledge about developmental milestones. The mean scores at T1, T2, and T3 doubled from the initial scores in the intervention group, while no difference was observed in the control group.

Conclusion: An interactive educational DVD can be an effective supplement to improve residents' knowledge of developmental milestones.

Key words: animated cartoons; developmental milestones; health communication; multimedia design; pediatric training

                    World J Pediatr 2011;7(2):161-166

  [Abstract] [Full Text] [PDF]  
Brief report:
Role of menthol in treatment of candidial napkin dermatitis
  Ali Mohammad Sabzghabaee, Firouzeh Nili, Alireza Ghannadi, Nastaran Eizadi-Mood, Maryam Anvari

Author Affiliations: Isfahan Clinical Toxicology Research Center, Isfahan University of Medical Sciences, Isfahan, Iran (Sabzghabaee AM, Eizadi-Mood N); Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran (Nili F); Department of Pharmacognosy, Isfahan University of Medical Sciences, Isfahan, Iran (Ghannadi A); Staff Pharmacist, Valiasr Pediatric Hospital, Tehran, Iran (Anvari M)

Corresponding Author: Nastaran Eizadi-Mood, MD, Associate Professor of Clinical Toxicology, Isfahan Clinical Toxicology Research Center, Isfahan University of Medical Sciences, Isfahan, Iran (Email: izadi@med.mui.ac.ir)

doi: 10.1007/s12519-011-0253-0

Background: The antibacterial, antifungal and probable anti-inflammatory effects of menthol were evaluated in the treatment of napkin dermatitis (ND).

Methods: A pilot clinical trial was conducted in Iran at the Tehran University of Medical Sciences. Eligible neonates with a diagnosis of candidial ND who did not require critical care or systematic antifungal and anti-inflammatory drugs were included in the study. Overall, 84 patients were randomly allocated into two groups: menthol group (n=42) receiving standard therapy (topical clotrimazole) plus menthol drops applied topically and control group (n=42) receiving standard therapy plus a placebo. Thirty-five neonates in each group finished the course of study and were analyzed for skin rash using Munz and Concannon rash scoring methods before therapy and on the 1st, 3rd, 5th and 7th day after the treatments.

Results: Demographic data and the baseline total skin rash score were not significantly different between the menthol and control groups. The total course of therapy for complete healing was found to be shorter in the menthol group (4.3±1.6 vs. 6.9±1.8 days, P=0.0001) and erythema and pustules had a significant (P=0.0001) relief in this group. During the study no severe adverse effects of the drug were observed.

Conclusion: Topical application of menthol may be effective in treatment of candidial ND.

Key words: clinical trial; menthol; napkin dermatitis

World J Pediatr 2011;7(2):167-170

  [Abstract] [Full Text] [PDF]  
Clinical practice:
Use of a silicone patch in the management of severe ischemic small bowel volvulus
  A Dahl-Farhoumand, J Birraux, A Darani, BE Wildhaber
  [Abstract] [Full Text] [PDF]  
Case reports:
Primary hyperoxaluria in a compound heterozygote infant
  Juan Mayordomo-Colunga, Debora Riverol, Eduardo Salido, Fernando Santos

Author Affiliations: Pediatric Nephrology, Department of Pediatrics, Hospital Universitario Central de Asturias & University of Oviedo, Oviedo, Spain (Mayordomo-Colunga J, Santos F); Research Unit, Hospital Universitario Canarias & University La Laguna, Tenerife, Spain (Riverol D, Salido E)

Corresponding Author: Juan Mayordomo-Colunga, MD, Servicio de Pediatría, Hospital Universitario Central de Asturias, Celestino Villamil, s/n. 33006 Oviedo, Spain (Tel: +34 636717615; Fax: +34 985107853; Email: jmcolunga@hotmail.com)

doi: 10.1007/s12519-010-0214-z

Background: Primary hyperoxaluria type 1 is a rare disorder caused by a defect in the hepatic metabolism of glyoxylate. Cases presenting in infancy are very uncommon and often have a severe course leading to early end-stage renal failure.

Methods: We treated a case of early presentation of primary hyperoxaluria type 1 and reviewed the relevant literature.

Results: A 4-month-old female infant was admitted to our hospital because of acute renal failure and nephrocalcinosis. Mutational analysis of alanine-glyoxylate aminotransferase gene revealed compound heterozygosity in the infant, confirming the development of primary hyperoxaluria type 1. A few weeks later, the condition of the infant worsened during an interdialytic period and died.

Conclusions: Interest of this case is based on the coexistence of two mutations of alanine-glyoxylate aminotransferase gene recently reported, and it confirms the severe course of the disease when it presents in infancy. It also highlights the importance of the association of nephrocalcinosis and urolithiasis as key diagnostic manifestations of primary hyperoxaluria type 1.

Key words: calcium oxalate; nephrocalcinosis;  primary hyperoxaluria; renal failure

                   World J Pediatr 2011;7(2):173-175

  [Abstract] [Full Text] [PDF]  
Follicular bronchiolitis in a child
  Yu-Wen Dai, Long Lin, Hong-Feng Tang, Ke-Wen Jiang

Author Affiliations: Department of Nephrology (Dai YW), Department of Pathology (Lin L, Tang HF), and Department of Laboratory (Jiang KW), Children's Hospital of Zhejiang University School of Medicine, Hangzhou, 310003, China

Corresponding Author: Ke-Wen Jiang, Department of Laboratory, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, 310003, China (Email: kewenjiang@gmail.com)

doi: 10.1007/s12519-011-0277-5

Background: Follicular bronchiolitis (FB) is an uncommon but important pulmonary manifestation in children.

Methods: In this report, we present the clinical presentations and histopathological data of an 8-year-old boy with FB.

Results: The patient had a history of recurrent cough and dyspnea for 5 years with progressive worsening of symptoms. An initial pulmonary function test showed an obstructive ventilatory defect. Chest X-ray demonstrated miliary nodules. High-resolution computed tomography showed reticulonodular opacification and central consolidation. Histopathological examination revealed that lymphoid follicles with reactive germinal centers distributed along the bronchioles. The boy responded favorably to corticosteroid therapy and recovered well.

Conclusions: Diagnosis of FB should be considered when a child presents with chronic bronchial obstruction. Open lung biopsy is necessary for confirmation of the diagnosis.

Key words: diagnosis; follicular bronchiolitis; histopathology

World J Pediatr 2011;7(2):176-178

  [Abstract] [Full Text] [PDF]  
Recurrent bacterial meningitis caused by an occult basilar skull fracture
  Xiao-Lu Chen, Li Jiang

Author Affiliations: Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing 400014, China (Chen XL, Jiang L)

Corresponding Author: Li Jiang, Department of Neurology, Children's Hospital of Chongqing Medical University, 136# Zhongshan 2 Road, Chongqing 400014, China (Tel: 86-23-63624424; Fax: 86-23-63622754; Email: neurojiang@gmail.com)

doi: 10.1007/s12519-010-0215-y

Background: We present a rare case of recurrent bacterial meningitis caused by an occult basilar skull fracture.

Methods: A 9-year-old boy presented with acute headache, fever and vomiting. He had recurrent meningitis episode one month earlier and a head trauma 6 months ago. Laboratory findings and lumbar puncture suggested an intracranial bacterial infection. Computerized tomography, magnetic resonance imaging and nasal endoscopy failed to find the presence of rhinorrhea. Spiral computed tomography was performed and a three-dimensional reconstruction of the bony cranium was done.

Results: A diagnosis of bone defect in the ethomoid was made. An endoscopic operation was performed to repair the defect successfully and the child was completely normal during a 4-month follow-up.

Conclusions: The skull base should be evaluated radiologically to find one or multiple bony defects in case of recurrent meningitis in absence of cerebrospinal fluid rhinorrhea. Compared to other neuroradiological technologies, three-dimensional computed tomography provides a better three-dimensional definition of the basilar skull fracture for both diagnosis and surgical planning. When the fracture is located in the anterior skull base, an endoscopic transnasal approach is considered the best option.

Key words: bacterial meningitis; basilar skull fracture; cerebrospinal fluid rhinorrhea; spiral computed tomography

World J Pediatr 2011;7(2):179-181

  [Abstract] [Full Text] [PDF]  
Late clinical manifestations of mitral valvedisease and severe pulmonary hypertension closure of foramen ovale during fetal life
  Yoichi Iwamoto, Akiko Tamai, Hidenori Kawasaki, Mio Taketazu, Hideaki Senzaki

Author Affiliations: Department of Pediatric Cardiology and Pediatrics, Saitama Medical University, Saitama, Japan (Iwamoto Y, Tamai A, Kawasaki H, Taketazu M, Senzaki H)

Corresponding Author: Hideaki Senzaki, MD, Staff Office Building 303, Department of Pediatric Cardiology, International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama, 350-1298 Japan (Tel: +81-42-984-4569; Fax: +81-42-984-4569; Email: hsenzaki@saitama-med.ac.jp)

doi: 10.1007/s12519-011-0276-6

Background: The patency of foramen ovale (FO) in fetal circulation is very important, and premature closure of FO could be associated with several pathological conditions.

Methods: We report a patient in whom premature closure of FO in fetal life was associated with late clinical onset of mitral valve stenosis and subsequent development of irreversible pulmonary hypertension (PH).

Results: The patient showed persistent PH after birth, which completely regressed at the age of 8 months. However, the patient developed heart failure due to mitral valve lesions (hammock valve) at the age of 11 months and underwent artificial valve replacement. The patient subsequently developed severe PH, which was refractory to anti-PH therapy with sildenafil and bosentan in addition to home oxygen.

Conclusions: This case illustrates that mitral stenosis can be overlooked during early neonatal life, and thus emphasizes the need for close follow-up for potential existence of mitral stenosis and later clinical manifestation in patients with premature FO closure even when initial careful examination of the mitral valves does not indicate any abnormalities. In addition, premature closure of FO could cause pulmonary vascular disease, which may lead to later development of irreversible PH.

Key words: foramen ovale; mitral valve; premature closure; pulmonary hypertension

                   World J Pediatr 2011;7(2):182-184

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