Pediatric Reports

513 KiB

Open AccessCase Report

Trabecular Variant of Juvenile Aggressive Ossifying Fibroma of Anterior Mandible

by Manjunatha B. S., Nagarajappa Das, Saraswati Naik and Gowramma R.

Pediatr. Rep. 2012, 4(2), e24; https://doi.org/10.4081/pr.2012.e24 - 19 Jun 2012

Cited by 4 | Viewed by 1

Juvenile ossifying fibroma (JOF) is an expansile intra-osseous lesion of the jaw that emulate odontogenic lesions frequently seen in patients under 15 years of age. They are histologically characterized by the presence of fibrous stromal cells along with mineralized tissues. Clinically, these are [...] Read more.

Juvenile ossifying fibroma (JOF) is an expansile intra-osseous lesion of the jaw that emulate odontogenic lesions frequently seen in patients under 15 years of age. They are histologically characterized by the presence of fibrous stromal cells along with mineralized tissues. Clinically, these are characterized by early age of onset, histological patterns, high rate of recurrence and the aggressive local behavior. The differential diagnosis of JOF with other fibro-osseous lesions of the jaw should be made along with an essential microscopic examination and be largely based on the character of the calcified products of the tumor. The purpose of this article is to present a rare clinical case of the trabecular type of JOF and to describe its clinical, radiological and histological characteristics. The clinician should be aware of this type of lesion in order to be able to distinguish this it from other fibrous lesions if encountered in routine practice and for appropriate treatment to be carried out. Full article

318 KiB

Open AccessArticle

Microfracture for Treatment of Knee Cartilage Defects in Children and Adolescents

by Gian M. Salzmann, Bert-Ram Sah, Hagen Schmal, Philip Niemeyer and Norbert P Sudkamp

Pediatr. Rep. 2012, 4(2), e21; https://doi.org/10.4081/pr.2012.e21 - 19 Jun 2012

Cited by 22 | Viewed by 1

Abstract

Even though operative microfracture is the most frequent method for treatment of limited knee joint cartilage lesions among adults, data about ouctome in children and adolescents are rare. We performed a retrospective chart review and telephone interview to analyze for the clinical outcome [...] Read more.

Even though operative microfracture is the most frequent method for treatment of limited knee joint cartilage lesions among adults, data about ouctome in children and adolescents are rare. We performed a retrospective chart review and telephone interview to analyze for the clinical outcome following knee joint cartilage defect microfracturing among 10 children. Mean postoperative Lysholm was 92.1±9.9 and Tegner was 7.0±1.9. Clinical outcome differed across knee joint regions, as well as in dependence of varying pre-operative symptom duration, although this was not significant. Regression analysis did not reveal a significant impact of patient or defect characteristics on clinical outcome. Arthroscopic microfracturing for treatment of limited size symptomatic knee joint cartilage defects among children and adolescents is considered a reasonable surgical option. However, long-term outcome and larger patient cohorts are required. Full article

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Open AccessCase Report

Bilateral Ultrasound Transversus Abdominis Plane Block in A Patient Affected from Menkes Disease

by Dario Galante, Marco Caruselli, Francesco Dones, Salvatore Meola, Gianluca Russo, Giuseppe Pellico, Antonio Caso, Massimo Lambo, Flora Donadei and Giuseppe Mincolelli

Pediatr. Rep. 2012, 4(2), e23; https://doi.org/10.4081/pr.2012.e23 - 13 Jun 2012

Cited by 1 | Viewed by 1

Abstract

This case report demonstrates the utility and safety of ultrasound transversus abdominis plane (TAP) block in a paediatric patient suffering from Menkes disease. Anaesthetists, and particularly paediatric and neonatal anaesthetists, have to make a careful assessment of these patients, who are subjected to [...] Read more.

This case report demonstrates the utility and safety of ultrasound transversus abdominis plane (TAP) block in a paediatric patient suffering from Menkes disease. Anaesthetists, and particularly paediatric and neonatal anaesthetists, have to make a careful assessment of these patients, who are subjected to frequent surgeries, despite their tender years. These operations are often necessary in order to improve the patient’s quality of life as much as possible. The choice of anaesthetic technique must take the particular nature of the disease and the patient’s medical history into consideration, while careful preparation and preoperative evaluations make it possible to administer the general or local anaesthetic safely. Official literature on the subject does not provide sufficient information about the use of local anaesthetic techniques. Moreover, the use of ultrasound TAP block in patients affected by Menkes disease has never been published in literature. In our experience, ultrasound TAP block has provided positive results, although the rarity of this childhood disease makes a detailed study difficult. Full article

231 KiB

Open AccessArticle

Treatment of Foodborne Botulism in Current Clinical Toxicology

by Afshin Mohammad Alizadeh and Nasim Zamani

Pediatr. Rep. 2012, 4(2), e22; https://doi.org/10.4081/pr.2012.e22 - 11 Jun 2012

Cited by 2 | Viewed by 1

Abstract

We read with interest the case presented byLonati D. et al.entitled Fatal course of food-borne botulism in an eight-month old infantbeing published in one of the recent issues ofPediatr Report.[...] Full article

308 KiB

Open AccessArticle

The Prune Belly Syndrome: Urological Aspects and Long-Term Outcomes of a Rare Disease

by Vahudin Zugor, Günter E. Schott and Apostolos P. Labanaris

Pediatr. Rep. 2012, 4(2), e20; https://doi.org/10.4081/pr.2012.e20 - 04 Jun 2012

Cited by 44 | Viewed by 1

Abstract

Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical [...] Read more.

Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%), malformations of the prostate in 3 (18.8%), urethral malformations in 8 (50%) and mega-ureter in 14 patients (87.5%). A mega-bladder was observed in 13 patients (81.3%). Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8%) and hydronephrosis in 9 patients (56.3%), respectively. Abdominoplasty was performed on 4 patients (25%). Urethral surgery was performed in 10 patients (62.5%). Seven patients (43.8%) required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor. Full article

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Open AccessCase Report

The Spectrum of Non-Classical Diamond-Blackfan Anemia: A Case of Late Beginning Transfusion Dependency Associated to a New RPL5 Mutation

by Piero Farruggia, Paola Quarello, Emanuela Garelli, Olivia Paolicchi, Giovanni Battista Ruffo, Liana Cuccia, Sonia Cannella, Giuseppa Bruno and Paolo D'Angelo

Pediatr. Rep. 2012, 4(2), e25; https://doi.org/10.4081/pr.2012.e25 - 26 Apr 2012

Cited by 4 | Viewed by 2

Abstract

Diamond Blackfan anemia typically presents in infants and is often associated with many kinds of malformations. Severity of anemia often needs transfusional support in the first months of life. We describe here a patient with Diamond Blackfan anemia related to a RPL5 mutation. [...] Read more.

Diamond Blackfan anemia typically presents in infants and is often associated with many kinds of malformations. Severity of anemia often needs transfusional support in the first months of life. We describe here a patient with Diamond Blackfan anemia related to a RPL5 mutation. The patient had no physical abnormalities and experienced a very late onset of transfusion dependency. Full article

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Open AccessReview

Out-of-Hospital Noninvasive Ventilation: Epidemiology, Technology and Equipment

by John Scott Baird and Thyyar M. Ravindranath

Pediatr. Rep. 2012, 4(2), e17; https://doi.org/10.4081/pr.2012.e17 - 24 Apr 2012

Cited by 7 | Viewed by 1

Abstract

Noninvasive ventilation has been utilized successfully in the pre- and out-of-hospital settings for a variety of disorders, including respiratory distress syndrome in neonates, neurologic and pulmonary diseases in infants and children, and heart failure as well as chronic obstructive pulmonary disease in adults. [...] Read more.

Noninvasive ventilation has been utilized successfully in the pre- and out-of-hospital settings for a variety of disorders, including respiratory distress syndrome in neonates, neurologic and pulmonary diseases in infants and children, and heart failure as well as chronic obstructive pulmonary disease in adults. A variety of interfaces as well as mechanical positive pressure devices have been used: simple continuous positive airway pressure devices are available which do not require sophisticated equipment, while a broad spectrum of ventilators have been used to provide bilevel positive airway pressure. Extensive training of transport teams may be important, particularly when utilizing bilevel positive airway pressure in infants and children. Full article

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Open AccessArticle

Non-Invasive Ventilation in Acute Respiratory Failure in Children

by Clara Abadesso, Pedro Nunes, Catarina Silvestre, Ester Matias, Helena Loureiro and Helena Almeida

Pediatr. Rep. 2012, 4(2), e16; https://doi.org/10.4081/pr.2012.e16 - 10 Apr 2012

Cited by 60 | Viewed by 1

Abstract

The aim of this paper is to assess the clinical efficacy of non-invasive ventilation (NIV) in avoiding endotracheal intubation (ETI), to demonstrate clinical and gasometric improvement and to identify predictive risk factors associated with NIV failure. An observational prospective clinical study was carried [...] Read more.

The aim of this paper is to assess the clinical efficacy of non-invasive ventilation (NIV) in avoiding endotracheal intubation (ETI), to demonstrate clinical and gasometric improvement and to identify predictive risk factors associated with NIV failure. An observational prospective clinical study was carried out. Included Patients with acute respiratory disease (ARD) treated with NIV, from November 2006 to January 2010 in a Pediatric Intensive Care Unit (PICU). NIV was used in 151 patients with acute respiratory failure (ARF). Patients were divided in two groups: NIV success and NIV failure, if ETI was required. Mean age was 7.2±20.3 months (median: 1 min: 0,3 max.: 156). Main diagnoses were bronchiolitis in 102 (67.5%), and pneumonia in 44 (29%) patients. There was a significant improvement in respiratory rate (RR), heart rate (HR), pH, and pCO2 at 2, 6, 12 and 24 hours after NIV onset (P<0.05) in both groups. Improvement in pulse oximetric saturation/ fraction of inspired oxygen (SpO2/FiO2) was verified at 2, 4, 6, 12 and 24 hours after NIV onset in the success group (P<0.001). In the failure group, significant SpO2/FiO2 improvement was only observed in the first 4 hours. NIV failure occurred in 34 patients (22.5%). Risk factors for NIV failure were apnea, prematurity, pneumonia, and bacterial co-infection (P<0.05). Independent risk factors for NIV failure were apneia (P<0.001; odds ratio 15.8; 95% confidence interval: 3.42-71.4) and pneumonia (P<0.001, odds ratio 31.25; 95% confidence interval: 8.33-111.11). There were no major complications related with NIV. In conclusion this study demonstrates the efficacy of NIV as a form of respiratory support for children and infants with ARF, preventing clinical deterioration and avoiding ETI in most of the patients. Risk factors for failure were related with immaturity and severe infection. Full article

454 KiB

Open AccessReview

Update on Perioperative Management of the Child with Asthma

by Francesco Dones, Grazia Foresta and Vincenzo Russotto

Pediatr. Rep. 2012, 4(2), e19; https://doi.org/10.4081/pr.2012.e19 - 05 Apr 2012

Cited by 10 | Viewed by 1

Abstract

Asthma represents the leading cause of morbidity from a chronic disease among children. Dealing with this disease during the perioperative period of pediatric surgical procedures is, therefore, quite common for the anesthesiologist and other professionalities involved. Preoperative assessment has a key role in [...] Read more.

Asthma represents the leading cause of morbidity from a chronic disease among children. Dealing with this disease during the perioperative period of pediatric surgical procedures is, therefore, quite common for the anesthesiologist and other professionalities involved. Preoperative assessment has a key role in detecting children at increased risk of perioperative respiratory complications. For children without an optimal control of symptoms or with a recent respiratory tract infection elective surgery should be postponed, if possible, after the optimization of therapy. According to clinical setting, loco-regional anesthesia represents the desirable option since it allows to avoid airway instrumentation. Airway management goals are preventing the increase of airflow resistance during general anesthesia along with avoiding triggers of bronchospasm. When their use is possible, face mask ventilation and laringeal mask are considered more reliable than tracheal intubation for children with asthma. Sevoflurane is the most commonly used anesthetic for induction and manteinance. Salbutamol seems to be useful in preventing airflow resistance rise after endotracheal intubation. Mechanical ventilation should be tailored according to pathophysiology of asthma: an adequate expiratory time should be setted in order to avoid a positive end-expiratory pressure due to expiratory airflow obstruction. Pain should be prevented and promptly controlled with a loco-regional anesthesia technique when it is possible. Potential allergic reactions to drugs or latex should always be considered during the whole perioperative period. Creating a serene atmosphere should be adopted as an important component of interventions in order to guarantee the best care to the asthmatic child. Full article

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Open AccessCase Report

Concurrence of Thyrotoxicosis and Gitelman’s Syndrome-Associated Hypokalemia-Induced Periodic Paralysis

by Shinsaku Imashuku, Tomoko Teramura-Ikeda, Naoko Kudo, Shigehiro Kaneda and Toshihiro Tajima

Pediatr. Rep. 2012, 4(2), e18; https://doi.org/10.4081/pr.2012.e18 - 02 Apr 2012

Cited by 8 | Viewed by 1

Abstract

A 16-year-old Japanese boy with a history of truancy had been treated at a psychiatric clinic. When the patient was referred to us for hypokalemia-associated paralysis, the diagnosis of thyrotoxic hypokalemic periodic paralysis was made, common in Asian men. Subsequently, the patient was [...] Read more.

A 16-year-old Japanese boy with a history of truancy had been treated at a psychiatric clinic. When the patient was referred to us for hypokalemia-associated paralysis, the diagnosis of thyrotoxic hypokalemic periodic paralysis was made, common in Asian men. Subsequently, the patient was found to have persistently high plasma renin and aldosterone levels. Thus, solute carrier family 12 member 3 gene (SLC12A3) analysis was performed. A novel missense homozygous mutation CTC->CAC at codon 858 (L858H) was found for which the patient was homozygous and his non-consanguineous parents heterozygote. These findings indicated that the patient developed hypokalemia-associated paralysis concurrently with thyrotoxicosis and Gitelman’s syndrome. This case underscores the importance of careful examinations of adolescents with complaints of truancy as well as of precise determinations of the causes of hypokalemia-associated paralysis. Full article

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Pediatr. Rep., Volume 4, Issue 2 (April 2012) – 10 articles

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