http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2013 , Hindawi Publishing Corporation . All rights reserved. Mon, 20 May 2013 14:56:44 +0000 http://www.hindawi.com/crim/pediatrics/2013/623102/ Pulmonary sequestration is a congenital abnormality consisting of a mass of pulmonary tissue that presents an abnormal connection with the tracheobronchial tree, with a blood supply coming from an anomalous artery derived from the systemic circulation. Extralobar pulmonary sequestration is characterized by having pleural coverings that are independent of the normal lungs, with vascular supply usually coming from the aorta or from one of its branches. This diagnosis can be suspected prenatally if an abdominal mass, generally below the diaphragm, is seen. Here, we present a case of a neonate on the second day of life, with ultrasonography showing extralobar pulmonary sequestration located above the left adrenal gland that prenatally simulated a neuroblastoma. Claudio Rodrigues Pires, Adriano Czapkowski, Edward Araujo Júnior, and Sebastião Marques Zanforlin Filho Copyright © 2013 Claudio Rodrigues Pires et al. All rights reserved. Thu, 16 May 2013 16:56:09 +0000 http://www.hindawi.com/crim/pediatrics/2013/607678/ Congenital fiber type disproportion (CFTD) is a form of congenital myopathy, which is defined by type 1 myofibers that are 12% smaller than type 2 myofibers, as well as a general predominance of type 1 myofibers. Conversely, myasthenia gravis (MG) is an acquired immune-mediated disease, in which the acetylcholine receptor (AChR) of the neuromuscular junction is blocked by antibodies. Thus, the anti-AChR antibody is nearly specific to MG. Herein, we report on a case of CFTD with increased anti-AChR antibody levels. A 23-month-old boy exhibited muscle hypotonia and weakness. Although he could walk by himself, he easily fell down and could not control his head for a long time. His blood test was positive for the anti-AChR antibody, while a muscle biopsy revealed characteristics of CFTD. We could not explain the relationship between MG and CFTD. However, we considered different diagnoses aside from MG, even when the patient’s blood is positive for the anti-AChR antibody. Shigemi Kimura, Shiro Ozasa, Keiko Nomura, Hirofumi Kosuge, and Kowasi Yoshioka Copyright © 2013 Shigemi Kimura et al. All rights reserved. Thu, 16 May 2013 10:29:57 +0000 http://www.hindawi.com/crim/pediatrics/2013/207907/ Dexmedetomidine is a highly selective α2-adrenoceptor agonist used for sedation due to its anxiolytic and analgesic properties without respiratory compromise. Due to its structural similarity to etomidate, there has been concern that dexmedetomidine may cause adrenal insufficiency. This concern was initially supported by animal studies, but subsequent human studies demonstrated mixed results. We describe the case of transient adrenal insufficiency in a 1-year-old male who presented with 24% total body surface 2nd degree burns. He required sedation with a prolonged, high-dose dexmedetomidine infusion with a peak infusion dose of 2.7 mcg/kg/hr and duration of 6.5 days. The patient developed lethargy and hypotension four days after discontinuation of his infusion. He had a random cortisol level which was low at 0.4 mcg/dL, and the concern for adrenal suppression was confirmed with an ACTH stimulation test with the baseline cortisol of 0.4 mcg/dL and inappropriate 60 minute post-ACTH stimulation cortisol of 7.8 mcg/dL. While further studies will be needed to clarify the risk of adrenal suppression secondary to dexmedetomidine, this case suggests that caution should be taken when administering dexmedetomidine to pediatric patients and highlights the need for future studies to look at appropriate dosing and duration of dexmedetomidine infusions. Elizabeth W. Tucker, David W. Cooke, Sapna R. Kudchadkar, and Sybil Ann Klaus Copyright © 2013 Elizabeth W. Tucker et al. All rights reserved. Thu, 16 May 2013 08:54:51 +0000 http://www.hindawi.com/crim/pediatrics/2013/764659/ The Ehlers Danlos syndromes (EDS) comprise a group of connective tissue disorders characterized by tissue fragility of the skin, ligaments, blood vessels and internal organs. Variable degrees of clinical severity and organ involvement are due to the molecular and biochemical heterogeneity of this group of disorders and have led to classification into well-characterized subtypes that are extending with the discovery of new genes and overlapping syndrome. Types include classical EDS (EDS I/II), hypermobility EDS (EDS III), vascular EDS (EDS IV), kyphoscoliosis EDS (EDS VI), arthrochalasia (EDS VIIA, B) and Dermatospraxis (EDS VIIC). Even to the well trained professional, the diagnosis of EDS remains a challenge due to overlapping symptoms and cases can remain without a well-defined classification. Life altering complications of this group of disorders include vascular and hollow organ rupture and ligamentous laxity leading to chronic dislocation with ensuing pain and long term disability. Patients initially present to the general practitioner who is expected to recognize the symptoms of EDS and to proceed with appropriate referral for definitive diagnosis and management to prevent devastating complications. In this paper, we describe a male with classical EDS complicated by devastating vascular and orthopedic events. Amel Karaa and Joan M. Stoler Copyright © 2013 Amel Karaa and Joan M. Stoler. All rights reserved. Wed, 08 May 2013 10:40:21 +0000 http://www.hindawi.com/crim/pediatrics/2013/307205/ Ultrasonographic (US) evaluation of spinal anomalies is limited. MRI is increasingly being used in the diagnosis of spinal anomalies. MRI has been able to show clearly the detailed anatomy of this rare case of type II diastematomyelia with triple splitting of the cord. Emad Sadek Shatla, El Said M. A. Bedair, and Ashraf Soliman Copyright © 2013 Emad Sadek Shatla et al. All rights reserved. Sat, 27 Apr 2013 15:42:53 +0000 http://www.hindawi.com/crim/pediatrics/2013/764216/ Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP. Saber Hammami, Khaled Harrathi, Khaled Lajmi, Samir Hadded, Chebil Ben Meriem, and Mohamed Néji Guédiche Copyright © 2013 Saber Hammami et al. All rights reserved. Mon, 22 Apr 2013 11:53:02 +0000 http://www.hindawi.com/crim/pediatrics/2013/704532/ A case of an 11-year-old prepubertal girl with Fournier's gangrene is presented. The diagnosis was posed after the disease had progressed to the typical clinical picture of frank gangrene. A benign viral infection, common warts, possibly represents the initiating event of the development of this necrotizing soft tissue infection. The development of childhood Fournier's gangrene in association with viral infections has been reported in the past. An optimal outcome was achieved by aggressive antibiotic and surgical treatment despite the late diagnosis. In children, Fournier's gangrene develops in previously healthy individuals with benign diseases often representing the initiating events; thus, high index of suspicion is necessary for an early diagnosis that will lead to the optimal outcome. Maria Tsinti, Theophani Tsekoura, Evangelos Blevrakis, Ioannis Vlachakis, and Amalia Tsilimigaki-Christaki Copyright © 2013 Maria Tsinti et al. All rights reserved. Thu, 18 Apr 2013 12:13:14 +0000 http://www.hindawi.com/crim/pediatrics/2013/652043/ Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disorder defined by the combination of biochemical abnormalities, tubulointerstitial nephritis, and uveitis. We describe a 12-year-old female, presented with a ten-day history of fever, characterized by sudden onset and rapid spontaneous resolution in few hours, accompanied by shivering, extreme fatigue, and loss of appetite. Laboratory values were consistent with renal failure of tubular origin. Renal biopsy confirmed a tubulointerstitial nephritis, with acute tubulitis, polymorphonuclear infiltration, and microabscesses. The renal interstitium was occupied by a dense inflammatory infiltrate, consisting of lymphocytes, plasma cells, and neutrophils. Glomerular structures were preserved. Ophthalmological examination that suggested a previous asymptomatic bilateral uveitis and HLA typing (HLA-DQA1*0101/0201 and HLA-DQB1*0303/0503) further supported the suspect of TINU syndrome. TINU syndrome is probably an underdiagnosed disorder, responsible for many cases of idiopathic anterior uveitis in young patients, especially in those who have asymptomatic renal disease and when proper diagnostic tests are not performed at the time of presentation. Alessia Paladini, Vittorio Venturoli, Giovanni Mosconi, Loretta Zambianchi, Luigi Serra, and Enrico Valletta Copyright © 2013 Alessia Paladini et al. All rights reserved. Thu, 18 Apr 2013 12:08:41 +0000 http://www.hindawi.com/crim/pediatrics/2013/928910/ Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but potentially life-threatening syndrome characterized by skin rash, fever, lymph node enlargement, and involvement of internal organs. It is most commonly induced by aromatic anticonvulsants and antibiotics. Nonaromatic anticonvulsants are rarely encountered as the causes of DRESS syndrome. In the present report, three discrete cases with DRESS syndrome developing due to three antiepileptic drugs, including valproic acid (nonaromatic), carbamazepine (aromatic), and lamotrigine (aromatic), and their treatment modalities were aimed to be discussed in light of the literature. To the best of our knowledge, our cases are the first children to be treated with pulse methylprednisolone in the literature. Celebi Kocaoglu, Ceyda Cilasun, Ece Selma Solak, Gulcan S. Kurtipek, and Sukru Arslan Copyright © 2013 Celebi Kocaoglu et al. All rights reserved. Thu, 18 Apr 2013 09:29:56 +0000 http://www.hindawi.com/crim/pediatrics/2013/237935/ Emanuel syndrome (ES) is a rare anomaly characterized by a distinctive phenotype, consisting of characteristic facial dysmorphism, microcephaly, severe mental retardation, developmental delay, renal anomalies, congenital cardiac defects, and genital anomalies in boys. Here, we report a male neonate, with the classical features of Emanuel syndrome. Madan Gopal Choudhary, Prashant Babaji, Nitin Sharma, Dilip Dhamankar, Gururaj Naregal, and Vijay Sunil Reddy Copyright © 2013 Madan Gopal Choudhary et al. All rights reserved. Tue, 16 Apr 2013 15:40:11 +0000 http://www.hindawi.com/crim/pediatrics/2013/680208/ Henoch-Schönlein purpura (HSP) is a vasculitis of small-sized blood vessels, resulting from immunoglobulin-A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. The characteristic rash of HSP consists of palpable purpuric lesions 2 to 10 mm in diameter concentrating in the buttocks and lower extremities. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. This report describes a 4.5-year-old female patient with HSP associated with hemorrhagic bullous lesions. Celebi Kocaoglu, Ramazan Ozturk, Yasar Unlu, Fatma Tuncez Akyurek, and Sukru Arslan Copyright © 2013 Celebi Kocaoglu et al. All rights reserved. Tue, 16 Apr 2013 10:49:07 +0000 http://www.hindawi.com/crim/pediatrics/2013/218124/ We report a patient with Cat eye syndrome (CES) associated with anatomical asplenia. To the best of our knowledge, there have been no prior reports of this association. Screening for asplenia in CES is potentially important, as asplenia places patients at increased risk for life-threatening bacterial infections. Hence patients with CES without a spleen may require the same routine precautions as any other asplenic patients, with penicillin prophylaxis and immunizations to protect against encapsulated organisms such as Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis. DeepakBabu Chellapandian and Adele Schneider Copyright © 2013 DeepakBabu Chellapandian and Adele Schneider. All rights reserved. Tue, 09 Apr 2013 17:07:38 +0000 http://www.hindawi.com/crim/pediatrics/2013/978198/ Through a case study of a child with cystic fibrosis, the interactions among various domains of health have been discussed—namely, biomedical, physical, psychological/behavioural, and social. In pediatrics, development is another key domain relevant to the management of a chronic disease. An individualised management plan for this case has been outlined, and consideration of this framework may be worthwhile when managing other paediatric patients with chronic disease. Patient empowerment and parental education, as well as good co-ordination of health service delivery, are imperative to holistic patient care. Wing Lung Alvin So Copyright © 2013 Wing Lung Alvin So. All rights reserved. Tue, 09 Apr 2013 13:14:46 +0000 http://www.hindawi.com/crim/pediatrics/2013/604921/ Triple A syndrome is an autosomal recessive disorder characterized by the triad of adrenocorticotropic hormone resistant adrenal insufficiency, achalasia, and alacrima. Our aim was to describe the neuropsychological characteristics and the cooccurring psychopathological and neurological disorders in an Italian male child suffering from Triple A syndrome at the time of admission (T0) and after one year of follow-up (T1). Many difficulties were observed in the motor domain, as well as in manual dexterity and static/dynamic balance domains of the motor task over time. In sharp contrast with previous literature reports on frequent mild cognitive dysfunction in patients with Triple A syndrome, our child did not show any mental retardation. By contrast, he showed an average IQ at T0 with a slight improvement at T1. To our knowledge, this report is the first describing neuropsychological profile and co-occurring psychopathological problems in a child with Triple A syndrome. Considering that the Triple A syndrome is a progressive disorder which can take years to develop the full-blown clinical picture, these patients require periodical medical controls. Moreover, assessment of neuropsychological and psychopathological features should be performed in patients with this disease, in order to underline the variability of this syndrome. Luigi Mazzone, Valentina Postorino, Lavinia De Peppo, Lia Vassena, Laura Fatta, Marco Armando, Giuseppe Scirè, Marco Cappa, and Stefano Vicari Copyright © 2013 Luigi Mazzone et al. All rights reserved. Thu, 04 Apr 2013 15:13:33 +0000 http://www.hindawi.com/crim/pediatrics/2013/356031/ Nikki Rink, Martin Bitzan, Gus O'Gorman, Mato Nagel, Elena Torban, and Paul Goodyer Copyright © 2013 Nikki Rink et al. All rights reserved. Thu, 04 Apr 2013 09:45:24 +0000 http://www.hindawi.com/crim/pediatrics/2013/896014/ Systemic lupus erythematosus (SLE) is an autoimmune disease associated with high morbidity and mortality, often caused by infection. We report two patients with SLE who were treated with steroids and immunosuppressive medication and then developed invasive Group B Streptococcus (GBS) infections. While GBS infection is rare in the nonneonatal pediatric age group, GBS should be considered when treating SLE patients presenting with signs of infection. Dongning Wu, Kenneth Bromberg, and Roberto Jodorkovsky Copyright © 2013 Dongning Wu et al. All rights reserved. Wed, 03 Apr 2013 08:37:47 +0000 http://www.hindawi.com/crim/pediatrics/2013/572301/ The case of a 14-year-old female with ectopic atrial tachycardia who had been followed for a history of anxiety and depression is presented. The patient was admitted to the intensive care unit after she attempted suicide, at which point there was electrocardiographic evidence of the atrial arrhythmia. During subsequent invasive electrophysiology study, a focus near the tricuspid annulus was localized and targeted for ablation, followed by resolution of the psychiatric symptoms. The case highlights the overlap between psychiatric complaints and true cardiac arrhythmia. A review of the literature is presented, with a special emphasis on distinguishing these two entities as well as a synopsis of this uncommon arrhythmia in the pediatric population. Peter Chau and Jeremy Moore Copyright © 2013 Peter Chau and Jeremy Moore. All rights reserved. Wed, 27 Mar 2013 17:40:01 +0000 http://www.hindawi.com/crim/pediatrics/2013/373712/ Necrotizing fasciitis is a serious soft tissue infection rarely occurring in children after blunt trauma. Due to its high morbidity and mortality rates, a high index of suspicion is necessary for prompt diagnosis and treatment. We describe a 6-year-old Costa Rican girl who died secondary to multiple complications following a posttraumatic necrotizing fasciitis. Yohel Ocaña, Rolando Ulloa-Gutierrez, and Adriana Yock-Corrales Copyright © 2013 Yohel Ocaña et al. All rights reserved. Mon, 18 Mar 2013 11:45:31 +0000 http://www.hindawi.com/crim/pediatrics/2013/471294/ We present the case of a 7-year-old boy, who received acetaminophen for the treatment of hyperpyrexia, due to an infection of the superior airways. 13 mg/kg (260 mg) of acetaminophen was administered orally before bedtime, and together with the expected antipyretic effect, the boy experienced sleep disruption and proprioceptive delirium. The symptoms disappeared within one hour. In the following six months, acetaminophen was administered again twice, and the reaction reappeared with similar features. Potential alternative explanations were excluded, and analysis with the Naranjo algorithm indicated a “probable” relationship between acetaminophen and this adverse reaction. We discuss the potential mechanisms involved, comprising imbalances in prostaglandin levels, alterations of dopamine, and cannabinoid and serotonin signalings. Carla Carnovale, Marco Pozzi, Andrea Angelo Nisic, Elisa Scrofani, Valentina Perrone, Stefania Antoniazzi, Emilio Clementi, and Sonia Radice Copyright © 2013 Carla Carnovale et al. All rights reserved. Wed, 06 Mar 2013 10:18:20 +0000 http://www.hindawi.com/crim/pediatrics/2013/947401/ Bronchopulmonary foregut malformations (BPFMs) include a wide variety of malformations such as intralobar or extralobar pulmonary sequestration, foregut duplication cysts, and diverticula of the gastrointestinal or pulmonary tree (Srikanth et al., 1992). Those anomalies in which a tract between the respiratory and alimentary systems exists are termed communicating bronchopulmonary foregut malformations (CBPFMs). Most infants with CBPFMs suffer from respiratory distress, and an accurate diagnosis may be difficult to make at the patient’s initial presentation. Herein we report such a case which posed a diagnostic challenge to us. This baby however survived and is doing well on a 2-year followup. Amitava Sur, Syamal Kumar Sardar, and Anshuman Paria Copyright © 2013 Amitava Sur et al. All rights reserved. Mon, 04 Mar 2013 14:01:18 +0000 http://www.hindawi.com/crim/pediatrics/2013/458543/ Neurofibromatosis type 1 (NF1) is a relatively common autosomal dominant genetic disorder with a prevalence of 1 in 3,000 (0.03%) at birth. Clinical features are café-au-lait macules, intertriginous freckling, dermal neurofibroma, iris hamartoma (Lisch nodules), and learning disability. NF1 vasculopathy is a serious but underrecognized complication involving the cerebrovascular and cardiovascular systems. The incidence of hypertension in patients with NF1 is around 1% and is associated mainly with renal artery stenosis in children. Only a few cases of thoracic aortic coarctation in association with hypertension and neurofibromatosis have been reported. Here we describe the case of a 4-year-old girl who presented with NF1 and hypertension due to atypical coarctation of the thoracic aorta. The diagnosis of coarctation of the thoracic aorta at the Th5-to-Th6 level was made following catheterization with a pressure gradient of 40 mmHg. The patient underwent surgery comprising resection of the coarctation of the thoracic aorta and graft interposition. On the basis of our findings, annual assessment of blood pressure is advised for patients with NF1. Masato Kimura, Shuhei Kakizaki, Kengo Kawano, Shinichi Sato, and Shigeo Kure Copyright © 2013 Masato Kimura et al. All rights reserved. Thu, 28 Feb 2013 11:17:46 +0000 http://www.hindawi.com/crim/pediatrics/2013/292053/ A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult. Oguzhan Güven Gümüştaş, Murat Sanal, Osman Güner, and Volkan Tümay Copyright © 2013 Oguzhan Güven Gümüştaş et al. All rights reserved. Wed, 20 Feb 2013 12:34:55 +0000 http://www.hindawi.com/crim/pediatrics/2013/727824/ Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) is an emerging public health problem worldwide. Severe invasive infections have been described, mostly associated with the presence of Panton-Valentine leukocidin (PVL). In Portugal limited information exists regarding CA-MRSA infections. In this study we describe the case of a previously healthy 12-year-old female, sport athlete, who presented to the hospital with acetabulofemoral septic arthritis, myositis, fasciitis, acetabulum osteomyelitis, and pneumonia. The MRSA isolated from blood and synovial fluid was PVL negative and staphylococcal enterotoxin type P (SEP) and type L (SEL) positive, with a vancomycin MIC of 1.0 mg/L and resistant to clindamycin and ciprofloxacin. The patient was submitted to multiple surgical drainages and started on vancomycin, rifampicin, and gentamycin. Due to persistence of fever and no microbiological clearance, linezolid was started with improvement. This is one of the few reported cases of severe invasive infection caused by CA-MRSA in Portugal, which was successfully treated with linezolid. In spite of the severity of infection, the MRSA isolate did not produce PVL. Catarina Gouveia, Alexandra Gavino, Ons Bouchami, Maria Miragaia, Luis Varandas, Herminia de Lencastre, and Maria Joao Brito Copyright © 2013 Catarina Gouveia et al. All rights reserved. Tue, 12 Feb 2013 15:11:50 +0000 http://www.hindawi.com/crim/pediatrics/2013/549649/ We present a case of neonatal chlamydial pneumonitis to illustrate that a high index of suspicion is necessary to make the diagnosis so that treatment can be promptly instituted. The child was afebrile and the only symptom was a cough. The respiratory equations are calculated to understand the respiratory physiology. There was no overt abnormality with ventilation, oxygenation, compliance, resistance, or ventilation-perfusion mismatch despite radiographic abnormality. The literature is searched to review if treatment with a systemic macrolide antibiotic is needed in an otherwise asymptomatic neonate with chlamydial pneumonitis. Kam Lun Hon and Alexander K. C. Leung Copyright © 2013 Kam Lun Hon and Alexander K. C. Leung. All rights reserved. Mon, 11 Feb 2013 10:10:53 +0000 http://www.hindawi.com/crim/pediatrics/2013/736164/ An asymptomatic 5-year-old girl presented with bradycardia during a routine well-child visit. Further evaluation revealed profound sinus bradycardia, exercise-induced bidirectional ventricular tachycardia, and supraventricular tachycardia. An echocardiogram showed heavy trabeculations in the left ventricular myocardium. This patient’s presentation suggested catecholaminergic polymorphic ventricular tachycardia and left ventricular noncompaction. Genetic testing revealed mutations in the cardiac ryanodine receptor (RyR2), calsequestron (CASQ2), and titin (TTN). She was effectively treated with beta-blockade to suppress tachyarrhythmias and pacemaker implantation to treat her bradycardia. Kelly R. Egan, J. Carter Ralphe, Larry Weinhaus, and Kathleen R. Maginot Copyright © 2013 Kelly R. Egan et al. All rights reserved. Thu, 07 Feb 2013 18:17:43 +0000 http://www.hindawi.com/crim/pediatrics/2013/758171/ In Amyand’s hernia, the hernia sac contains the appendix. This can be normal and accidentally found during herniotomy or inflamed and present as acute scrotum, although the latter is less frequent. We present a case of a male infant with scrotal abscess resulting from Amyand’s hernia, with appendicitis and periappendicular abscess. Cláudia Piedade and Júlio Reis Alves Copyright © 2013 Cláudia Piedade and Júlio Reis Alves. All rights reserved. Wed, 06 Feb 2013 10:06:48 +0000 http://www.hindawi.com/crim/pediatrics/2013/931703/ Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and a leading cause of acquired heart disease. It is assumed that there is an activation of the immune system by an infectious trigger in a genetically susceptible host. Neuroblastoma is the most common extracranial solid tumor in young children. It mainly originates from primordial neural crest cells that generate the adrenal medulla and sympathetic ganglia. A diagnosis of concurrent KD and neuroblastoma in a living child has been made in only one previous report. We report the second case and review the literature. Samin Alavi, Alireza Fahimzad, Farzaneh Jadali, Farid Ghazizadeh, and Armin Rashidi Copyright © 2013 Samin Alavi et al. All rights reserved. Mon, 04 Feb 2013 16:28:41 +0000 http://www.hindawi.com/crim/pediatrics/2013/721871/ Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancreatitis should be ruled out in the context of vomiting in any patient with isovaleric acidemia. Conversely, branched-chain organic acidemias should be included in the differential diagnosis of any child with pancreatitis of unknown origin. Elpis Mantadakis, Ioannis Chrysafis, Emmanouela Tsouvala, Athanassios Evangeliou, and Athanassios Chatzimichael Copyright © 2013 Elpis Mantadakis et al. All rights reserved. Mon, 28 Jan 2013 14:34:21 +0000 http://www.hindawi.com/crim/pediatrics/2013/426923/ We present 3 patients with 4 causes of mediastinal fluid collection after congenital cardiac surgery in this extended case report. Volumetric computed tomography played an essential role in diagnosing causes and extent, relevant to subsequent management. Recent advances in volumetric computed tomography allow fast and accurate imaging of cardiovascular and extravascular structures in children with acceptable radiation dose, providing a powerful imaging tool for the evaluation of complications after congenital cardiac surgery. Arno A. W. Roest, Joost Roelofs, Mark G. Hazekamp, Marry E. B. Rijlaarsdam, Jacob Geleijns, and Lucia J. M. Kroft Copyright © 2013 Arno A. W. Roest et al. All rights reserved. Thu, 17 Jan 2013 14:53:34 +0000 http://www.hindawi.com/crim/pediatrics/2013/726826/ We present a case of a seven-year-old immunocompetent female patient who developed systemic symptoms mimicking an autoimmune rather than an infectious disease. The patient presented with rash, biquotidian fever, night sweats, and arthralgias. There was no antecedent history of cat contact. Investigations showed increased inflammatory markers, leukocytosis, thrombocytosis, hypercalcemia, and raised angiotensin-converting enzyme. Interferon-gamma releasing assay for tuberculosis infection was negative. Abdominal imaging demonstrated multifocal lesions of the liver and spleen (later proved to be granulomata), chest X-ray showed enlarged hilar lymph nodes, and ophthalmology review revealed uveitis. Clinical, laboratory, and imaging features pointed towards sarcoidosis. Subsequently, raised titers (IgM 1 : 32, IgG 1 : 256) against Bartonella confirmed the diagnosis of B. henselae infection. She was treated with gentamycin followed by ciprofloxacin; repeat investigations showed complete resolution of findings. The presence of hepatic and splenic lesions in children with bartonellosis is well documented. Our case, however, exhibited certain unusual findings such as the coexistence of acute ocular and systemic involvement in an immunocompetent host. Serological testing is an inexpensive and effective way to diagnose bartonellosis in immunocompetent patients; we suggest that bartonella serology is included in the baseline tests performed on children with prolonged fever even in the absence of contact with cats in countries where bartonellosis is prevalent. Despoina N. Maritsi, Diagoras Zarganis, Zoi Metaxa, Georgia Papaioannou, and George Vartzelis Copyright © 2013 Despoina N. Maritsi et al. All rights reserved.