The Journal of Pediatric Research

The Journal of Pediatric Research

Basic info

  • Publisher: Galenos Publishing House
  • Country of publisher: turkey
  • Date added to EuroPub: 2019/Aug/08

Subject and more

  • LCC Subject Category: Medicine, Pediatrics
  • Publisher's keywords: Medicine, Pediatric, Genetics, Allegy and Immunology, Hepatology and Nutrition, Infectious Diseases, Neurology, Oncology
  • Language of fulltext: english

Publication charges

  • Article Processing Charges (APCs): No
  • Submission charges: No
  • Waiver policy for charges? No

Editorial information

Open access & licensing

  • Type of License: CC BY-NC-ND
  • License terms
  • Open Access Statement: Yes
  • Year open access content began: 2014
  • Does the author retain unrestricted copyright? False
  • Does the author retain publishing rights? False

Best practice polices

  • Permanent article identifier: DOI
  • Content digitally archived in: Other
  • Deposit policy registered in: None

This journal has '15' articles

Pediatric Dentist-Pediatrician Cooperation in Early Diagnosis of Congenital Tooth Agenesis

Pediatric Dentist-Pediatrician Cooperation in Early Diagnosis of Congenital Tooth Agenesis

Authors: Akif Demirel, Şaziye Sarı
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Abstract

Early diagnosis of congenital tooth agenesis increases the quality of treatment options and the final success of the treatment. However, in some clinical conditions, overlooking this diagnosis causes late diagnosis of these anomalies. Late diagnosis reduces the number of appropriate treatments, increases the cost of treatment and may lead to possible complications. In the diagnosis of congenital tooth agenesis, findings during the routine medical examination of the pediatric patient are beneficial. At this point, as well as pediatric dentists, pediatricians also play a great role in early diagnosis of these anomalies. The aim of this study is to draw attention to pediatric dentist-pediatrician cooperation in the early diagnosis of congenital tooth agenesis.

Keywords: Pediatric dentist, pediatrician, early diagnosis, congenital tooth agenesis
Evaluation of Syringe Feeding Compared to Bottle Feeding for the Transition from Gavage Feeding to Oral Feeding in Preterm Infants

Evaluation of Syringe Feeding Compared to Bottle Feeding for the Transition from Gavage Feeding to Oral Feeding in Preterm Infants

Authors: Birgül Say, Mehmet Büyüktiryaki, Nilüfer Okur, Gülsüm Kadıoğlu Şimşek, Fuat Emre Canpolat, Nurdan Uraş, Şerife Suna Oğuz
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Abstract

Aim: Syringe feeding is a good alternative to a nursing supplementer when breastfeeding is not possible. Materials and Methods: This study was conducted at a level 2 neonatal intensive care unit in the Zekai Tahir Burak Maternity Teaching Hospital in a comparative and descriptive clinical study pattern. The study was carried out with 47 preterm infants in a syringe-fed group (SG) and 56 preterm infants in a bottle-fed group (BG). Primary outcomes were time of transition from gavage feeding to full oral feeding time of transition from tube to breastfeeding, and hospitalization time. Secondary outcomes were body weight at discharge (g), daily body weight gain (g/days) and gastro-intestinal intolerance symptoms during the transition period. Results: Mean gestational ages were 29.82±2.03 vs 28.18±1.56 weeks (p=0.24) and mean birth weights were 1,150.31±232.29 vs 1,016.87±186.64 g (p=0.72) in the SG and BG groups, respectively. One hundred and three infants receiving gavage feeding with gestational ages ranging from 26 to 32 weeks were evaluated for full oral feeding start time. Syringe-fed preterm infants had a mean of 40.45±19.50 days and bottle-fed infants had a mean of 53.81±16.97 days (p>0.05). The time to transition to breastfeeding (42.54±21.21 days) and time to discharge (54.48±26.92 days) in the SG was significantly shorter compared to the BG (50.45±15.95, 67.21±22.07, respectively) (p<0.05). Conclusion: We found that preterm infants for whom feeding with a syringe was used as a reinforcement in addition to orogastric feeding switched to full breastfeeding in a shorter time compared to infants who were fed by bottle. From these results, we recommend syringe feeding as a transitional method prior to breastfeeding for preterm infants during hospitalization.

Keywords: Preterm infants, exclusive breastfeeding, feeding method
Can Serial Measurement Enhance the Diagnostic Value of Procalcitonin as a Marker of Gram-negative Bacteremia in Children with Acute Leukemia?

Can Serial Measurement Enhance the Diagnostic Value of Procalcitonin as a Marker of Gram-negative Bacteremia in Children with Acute Leukemia?

Authors: Zümrüt Şahbudak Bal, Gülinaz Ercan, Deniz Yılmaz Karapınar
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Abstract

Aim: Despite improvements in diagnosis and treatment, infections are still major cause of morbidity and mortality in children with febrile neutropenia (FN). In these patients, due to inadequate inflammatory response and subtle clinical symptoms, to determine the source of infection can be challenging. Therefore, it is important to distinguish infections from other non-infectious causes, for both to choose appropriate antibiotic and to reduce the redundant antibiotic use. Materials and Methods: In this retrospective study, we aim to evaluate serial procalcitonin (PCT) levels for predicting bacteremia particularly caused by Gram-negative microorganism. Results: Among FN episodes caused by Gram-negative microorganism, the median level of second PCT sample obtained between 48 and 72 hours PCT 2 was found to be significantly higher when compared to infections caused by Coagulase-negative Staphylococcus or culture-negative confirmed infections, p value was 0.003; however, fever onset PCT 1 and C-reactive protein (CRP) 1 values showed no significant difference (p>0.05). The area under curve values demonstrated by receiver operating characteristic (ROC) analysis for CRP 1, CRP 2, PCT 1, PCT 2 were 0.664, 0.748, 0.504 and 0.842, respectively. Conclusion: This study showed that initial PCT levels were not significantly correlate with culture-confirmed bacterial infection. Therefore, initial PCT values do not help the clinicians in terms of administering or postponing empirical antibiotics at the time of fever onset. However, third day PCT levels present as a good diagnostic marker due to a higher sensitivity and specificity when comparing them to the initial values. Determination of serial PCT may enhance the diagnostic value of PCT diagnostic marker in FN episodes caused by Gram-negative bacteria with a high sensitivity (87.5%). This study also demonstrated that PCT could be used to rule out bacterial infections particularly caused by Gram-negative bacteria.

Keywords: Procalcitonin, febrile neutropenia, child, Gram-negative bacteremia
Can Temporomandibular Joint Disorders Be Diagnosed Beforehand by Assessment of Postural Irregularities?

Can Temporomandibular Joint Disorders Be Diagnosed Beforehand by Assessment of Postural Irregularities?

Authors: Canan Dağ, Akif Demirel, Nurhan Özalp
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Abstract

Aim: In many studies, the relationship between postural status and temporomandibular disorders (TMD) have been investigated, however there is no consensus on this regard. The aim of this study was to investigate the relationship between postural irregularities forward head posture (FHP) and different shoulder levels (DSL) and TMD prevalence in different dentition stages. Materials and Methods: This study, which included children between 4 and 14 years of age attending public schools in Ankara, was planned as a cross-sectional study. Temporomandibular joint (TMJ) was examined intra- and extra-orally. After the examination, the relationship between TMD and body posture (FHP and DSL) was investigated. For statistical analysis, chi-square test and Fisher’s exact test were used with significance level of p<0.05. Results: FHP and DSL were statistically related to TMD (p<0.05). In primary dentition, there was no significant relation between FHP and TMD, however, DSL were found to be significantly related to TMD (p<0.05). In mixed dentition, both of these parameters were statistically related to TMD (p<0.05). However, in the permanent dentition, there was no relationship between body posture and TMD. Conclusion: Since there is a strong correlation between postural irregularities and TMD especially in the mixed dentition stage, TMJ and postural status of pediatric patients should be examined as early as possible in the stages of the life. In this regard, the awareness of pediatricians and pediatric dentists to this matter needs to be improved.

Keywords: TMJ, TMD, head posture, body posture
Visceral Leishmaniasis in Children in Southern Turkey: Evaluation of Clinical and Laboratory Findings and Liposomal Amphotericin B Treatment

Visceral Leishmaniasis in Children in Southern Turkey: Evaluation of Clinical and Laboratory Findings and Liposomal Amphotericin B Treatment

Authors: Barbaros Şahin Karagün, Özlem Özgür, İlgen Şaşmaz, Bülent Antmen, Emine Kocabaş, Emre Alhan
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Abstract

Aim: Visceral leishmaniasis (VL) is a systemic infection that spreads hematogenously and affects the reticuloendothelial system by the infection of macrophages. VL occurs commonly in children, and only rarely in adults. VL should be considered in patients with prolonged high fever, hepatosplenomegaly, pancytopenia, weight loss, pallor and hypergammaglobulinemia. Materials and Methods: In this study, a total of 18 pediatric patients -9 (50%) males and 9 (50%) females- treated for VL at our clinic from January 2004 to July 2014 were analyzed retrospectively. Average time from symptom onset to hospital admission was 64±21 days (range: 30-100 days). The mean age of patients was 88±40 months (range: 36-182 months). Results: The most common symptom at presentation was fever (88.9%). Other common symptoms were fatigue, chills, weight loss and anorexia. Physical examination revealed splenomegaly and hepatomegaly in all patients. Anemia (92.4%), leukopenia (78.7%) and thrombocytopenia (76.2%) were the most prominent laboratory abnormalities and 82.2% of the patients were pancytopenic on admission. Bone marrow smear was positive for leishmania in 100% of the patients. All patients received treatment with liposomal amphotericin B. Conclusion: In certain regions, increased humidity rates associated with construction of dams and irrigation canals may lead to changes in the ecological balance and thus cause an increase in the population of disease-spreading vectors. Additionally, recent migration from the middle-eastern region to western parts of the world due to regional civil wars may have contributed to the observed increase in the incidence of various diseases such as VL.

Keywords: Leishmania, visceral leishmaniasis, liposomal Amphotericin B, child, Turkey
Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Pediatric Liver Transplantation Outcomes for Metabolic and Non-metabolic Diseases in Turkey: A Single Center Experience

Authors: Miray Karakoyun, Ebru Canda, Sirmen Kızılcan, Orkan Ergün, Murat Kılıç, Funda Çetin, Sema Aydoğdu
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Abstract

Aim: Liver transplantation (LT) is performed for several indications in the pediatric population, including malignancy and acute hepatic failure among others. LT has become an important treatment alternative for metabolic diseases. In most pediatric transplant centers, metabolic liver disease is the second most common indication for LT after biliary atresia. Our studies aim is to compare the post-transplant outcomes of those patients with metabolic and other liver diseases in our transplant program. Materials and Methods: One hundred eighty-nine patients who underwent LT between 1997 and 2015 due to metabolic diseases and acute or chronic liver failure were included in the study. This study was performed retrospectively. Results: We enrolled 189 patients in our study. 54% (n=102) male and 46% (n=87) female patients were included in the study. The metabolic disease group included 56 patients and the non-metabolic disease group contained 133 patients. Progressive familial intrahepatic cholestasis is the most common disease among metabolic diseases resulting in LT and Wilson disease is the second most common. Post-transplant immunosuppression was similar for both groups. There was no difference in both groups regarding the onset of post-transplant complications for graft type or recipient age. Biliary and portal vein complications were most particularly defined in the group with non-metabolic diseases. There was no significant difference in survival between the two groups. Conclusion: LT is an important treatment option for acute hepatic failure and end-stage liver diseases. In addition, LT is an alternative treatment option for some metabolic diseases.

Keywords: Complications, non-metabolic diseases, metabolic diseases, liver, survival, transplantation
Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis

Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis

Authors: Ebru Canda, Melis Köse, Mehtap Kağnıcı, Meral Dondurmacı, Sema Kalkan Uçar, Eser Sözmen, Reşit Ertürk Levent, Mahmut Çöker
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Abstract

Aim: Cardiovascular involvement is common in patients with mucopolysaccharidoses (MPS). In this study, we investigated the effects of the markers involved in vascular endothelial injury pathogenesis [transforming growth factor β- (TGF-β)], interleukin-6 (IL-6), IL-10, high sensitive-C reactive protein (hs-CRP), vascular endothelial growth factor (VEGF), N-terminal pro-Natriuretic peptide (NT-proBNP) and the clinical, laboratory and echocardiographic findings of the patients. Materials and Methods: A total of 37 patients (5 MPS I, 4 MPS II, 2 MPS IIIa, 4 MPS IIIb, 14 MPS IVa, 8 MPS VI) and 32 controls with similar age and sex were included in the study. Results: Corneal clouding was seen in 29 (78%) patients. There were 23 (62%) patients with organomegaly, and 28 (75%) patients with hearing loss. When the groups were compared in terms of NT-proBNP, hs-CRP, TGF-β, IL-6, IL-10 and VEGF levels, there was a statistically significant increase in the patient group for NT-proBNP and VEGF (p=0.04, p=0.03, respectively). The carotid intima media thickness was statistically significantly higher in the patient group (p<0.001). The left ventricular diastolic diameter was significantly higher in the patient group (p=0.009), intraventricular septum thickness was significantly higher in the patient group (p<0.001). The E/A ratio was significantly lower in the patient group (p<0.001). Conclusion: Cardiac involvement in MPS patients is a major cause of mortality and morbidity. It is thought that cytokines, proinflammatory markers are elevated in patients with vascular damage like other lysosomal diseases. There is a need for further studies to determine biomarkers for vascular involvement.

Keywords: Mucopolysaccharidoses, carotis intima media thickness, vascular involvement, cytokines
Sleep Characteristics of Pediatric Burn Patients

Sleep Characteristics of Pediatric Burn Patients

Authors: Esra Ardahan Akgül, Hatice Yıldırım Sarı
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Abstract

Aim: Soft tissue injuries which happen because of high heat, chemicals etc. are called burns. Sleep is a complex behavior regulated by the interaction of anatomical and neurochemical areas in the central nervous system. After being burnt; patients are exposed to many stressors that cause sleep interruption and decrease sleep quality. The purpose of this study was to determine sleep characteristics, sleeplessness and sleep habits in pediatric burn patients. Materials and Methods: This descriptive study was carried out on 96 children who were 2-6 years old in the pediatric surgery department/burn unit for a period of between 2-7 days. For data collection, a demographic data collection form and a sleep problems characteristics and identification survey, which was created by the researchers, were used. Data were collected via face to face interviews with mothers. Questions were asked in order to compare pre- and post- burn sleep quality. Results: Of the children, 53.1% were male and their mean age was 3.36±1.39 years. There was a significant difference between pre- and post- burn periods on children’s sleeping and waking hours, total and night sleep duration, total daily sleep duration, existence of sleep interruption and number of interruptions, trouble of falling asleep and sleep latency, being tired in the morning, forcing the children to wake up and also the duration of waking up. Conclusion: With the knowledge about the importance of sleep, attention should be paid to the sleep of children that we are providing care to. Supportive environmental regulations should be made to improve the quality of sleep in hospitals.

Keywords: Pediatrics, burn, sleep
Prevalence of Tonsillar Actinomycosis in Tonsillectomy Specimens; Clinical Importance and Management

Prevalence of Tonsillar Actinomycosis in Tonsillectomy Specimens; Clinical Importance and Management

Authors: Naeimeh Daneshmandan, Mohammadebrahim Yarmohammadi, Poopak Izadi
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Abstract

Aim: We aimed to assess the prevalence of actinomycosis in tonsillectomy specimens and its clinical importance and management in our center. Materials and Methods: In this retrospective matched case-control study, patients who underwent tonsillectomy during 2010-2015 in Shahid Mostafa Khomeini Hospital, Tehran, were studied. Data regarding age, sex, indication for surgery (such as recurrent tonsillitis or sleep-disordered breathing), tonsil size, and length of disease before surgery were collected in both histopathologically positive (case) and negative (control) patients for actinomycosis. Results: From the 260 studied tonsillectomy specimens, actinomycosis was found in 20 (8.75%) and 60 Actinomycosis negative cases were chosen randomly for the control group. There was no statistically significant difference between the groups with respect to age, sex, length of disease before surgery, and incidence of actinomycosis in tonsillectomy due to recurrent tonsillitis or sleep-disordered breathing. However, the tonsil size was significantly greater in specimens positive for actinomycosis (8.65±1.5 mL versus 4.38±0.22 mL, p<0.001). Conclusion: Actinomycosis in tonsillectomy specimens is an incidental histopathological finding and does not necessarily correlate with active disease; while it is associated with tonsillar hypertrophy and increased tonsil size.

Keywords: Actinomycosis, tonsillectomy, recurrent tonsillitis, tonsillar hypertrophy
Investigations of Microtubule-associated Protein 2 Gene Expression in Spinal Muscular Atrophy

Investigations of Microtubule-associated Protein 2 Gene Expression in Spinal Muscular Atrophy

Authors: Gamze Bora, Ceren Sucularlı, Niko Hensel, Peter Claus, Hayat Erdem Yurter
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Abstract

Aim: Spinal muscular atrophy (SMA) is a devastating genetic disease in childhood andff is caused by the absence of functional survival motor neuron (SMN) protein, which leads to impairments of the cytoskeleton, especially in neurons. Dysregulation of actin dynamics have been linked to SMA patho mechanisms, however involvement of altered microtubule dynamics is largely unknown. In this study, we investigated differentially expressed microtubule-related genes using in vitro and in vivo SMA model systems. Materials and Methods: By focusing on microtubule-related genes, we re-analyzed publically available gene expression arrays, which were previously performed with induced pluripotent stem cell-derived motor neurons of SMA patients and the spinal cords of SMA mice. We found altered expressions of microtubule-associated protein 2 (MAP2), which was validated by real time reverse-transcription polymerase chain reaction using the SMN knock-down NSC34 cell line and the severe SMA mouse model. Results: We showed that the expression of MAP2 gene was significantly upregulated in both expression arrays. Upregulation was also detected in the brain and spinal cord tissues of severe SMA mice at different developmental stages. Conclusion: Our findings suggest that microtubule regulatory proteins may be altered in SMN depleted cells and further research is needed to elucidate the contribution of dysregulated microtubule dynamics towards SMA.

Keywords: Spinal muscular atrophy, exon-array, microtubule-associated protein 2
A Novel Mutation in Fanconi Bickel Syndrome Diagnosed in the Neonatal Period

A Novel Mutation in Fanconi Bickel Syndrome Diagnosed in the Neonatal Period

Authors: Şükran Keskin Gözmen, Kıymet Çelik, Şebnem Çalkavur, Erkin Serdaroğlu
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Abstract

Fanconi Bickel Syndrome (FBS), also known as glycogen storage disease Type XI, is a rare autosomal recessive disorder. This syndrome has many different identified mutations and it is rarely diagnosed during the neonatal period. Our patient is a two-week old female newborn who was admitted to our hospital with fever and dehydration. Renal Fanconi Syndrome was diagnosed in the presence of polyuria, proteinuria, glycosuria, hyperchloremic metabolic acidosis with normal anion gap and positive urine anion gap, hyperuricemia, hypophosphatemia and an increased excretion of phosphorus in urine. A novel mutation, IVS8 homozygote g.24401-24406del6 in the GLUT2 gene was demonstrated by the Sanger method. The same mutation was detected as heterozygote in her parents. Although most of the affected infants have a consanguineous parentage history in the literature, our patient was born to non-consanguineous parents. Also, according to our knowledge, few FBS patients were diagnosed in the newborn period. Our patient was diagnosed with a novel mutation in her first month of life.

Keywords: Fanconi Bickel Syndrome, glycogen storage disease Type XI, mutation, neonatal period
Intrapulmonary Sequestration in a 2-month-old Baby Treated Successfully by a Combination of Embolization and Surgical Resection

Intrapulmonary Sequestration in a 2-month-old Baby Treated Successfully by a Combination of Embolization and Surgical Resection

Authors: Aykut Eşki, Gökçen Kartal Öztürk, Celal Çınar, Hakkı Ata Erdener, Emre Divarcı, Timur Meşe, Hüseyin Hüdaver Alper, Deniz Nart, Figen Gülen, Esen Demir
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Abstract

Intralobar pulmonary sequestration is a rare congenital malformation, usually diagnosed later in childhood or adolescence. We report a case who presented with tachypnea and was diagnosed at 2 months of age. Pulmonary sequestration is usually managed by embolization or surgical resection. Recently, preoperative embolization of aberrant arteries to minimize the risk of serious intraoperative hemorrhage has also been described. Our case was successfully treated with embolization followed by a thoracoscopic resection.

Keywords: Embolization, pulmonary sequestration, surgical resection
Hereditary Neuropathy with Liability to Pressure Palsy: A Case Diagnosed with a Quick Multiplex Ligation-dependent Probe Amplification Test

Hereditary Neuropathy with Liability to Pressure Palsy: A Case Diagnosed with a Quick Multiplex Ligation-dependent Probe Amplification Test

Authors: Seda Kanmaz, Erdem Şimşek, Hepsen Mine Serin, Murat Kadri Erdoğan, Sanem Yılmaz, Gül Aktan, Hasan Tekgül, Sarenur Gökben
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Abstract

Hereditary neuropathy with a liability to pressure palsies (HNPP) represented by recurrent focal pressure neuropathies is rare in childhood. Here we present a 10-year-old girl admitted to our hospital with a recurrent weakness in her foot and diagnosed as HNPP with a quick Multiplex Ligation-dependent Probe Amplification test revealing PMP22 deletion.

Keywords: Hereditary neuropathy, pressure neuropathy, mjultiplex ligation-dependent probe amplification
Plastic Bronchitis Following Fontan Procedure: A Case Report

Plastic Bronchitis Following Fontan Procedure: A Case Report

Authors: Eser Doğan, Derya Aydın, Zülal Ülger, Ertürk Levent
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Abstract

Fibrinous bronchitis, also known as plastic bronchitis or pseudomembranous bronchitis, usually manifests clinically with non-productive cough and dyspnea. Prior to the expectoration of plaque formations, patients have a history of viral or other respiratory disease. Plastic bronchitis is an extremely rare and fatal complication associated with Fontan procedure. The condition is characterized by the formation of inspissated bronchial casts which may cause life-threatening airway obstructions. Although the pathogenesis of this condition remains unclear, it is believed to involve elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage. A 9-year-old male patient with Down syndrome underwent Fontan procedure 29 months earlier due to complete endocardial cushion defect and single ventricular physiology. The patient presented to the emergency department due to sudden-onset respiratory distress and fever (38.5 °C). Bilateral diffuse sibilant rhonchi, secretory rales, and intercostal retractions were noted during pulmonary system examination. Posterior-anterior chest x-ray showed cardiomegaly (cardiothoracic ratio=0.6) and bilateral diffuse infiltration. While under treatment, the patient experienced sudden-onset cough with expectoration of rubbery sputum in the form of branching bronchi-shaped casts. Samples of the inspissated sputum were sent to the Pathology and Microbiology departments for examination. The pathology report indicated fibrinoid material composed of a small number of inflammatory cells and bacterial plaques. Normal bacterial flora was identified in microbiological culture. Clinical presentation can vary from mild clinical findings to life-threatening symptoms. As in our patient, the diagnosis is made clinically, based on expectoration of bronchial casts or their detection during bronchoscopy. As our patient responded well to medical treatment, we proceeded with clinical follow-up. Plastic bronchitis is a very rare entity, and carries a poorer prognosis when it develops after congenital heart disease, as in our case.

Keywords: Plastic bronchitis, fontan operation, fibrinous bronchitis

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