Nephrotic Syndrome Following H1N1 Influenza in a 3-Year-Old Boy

Background

The pandemic influenza A/H1N1, spread through the world in 2009, producing a serious epidemic in Italy. Complications are generally limited to patients at the extremes of age (<6 months or >65 years) and those with comorbid medical illness. The most frequent complications of influenza involve the respiratory system.

Case Presentation

A 3-year-old boy with a recent history of upper respiratory tract infection developed a nephrotic syndrome. Together with prednisone, furosemide and albumin bolus, a therapy with oseltamivir was started since the nasopharyngeal swab resulted positive for influenza A/H1N1. Clinical conditions and laboratory findings progressively improved during hospitalization, becoming normal during a 2 month follow up.

Conclusion

The possibility of a renal involvement after influenza A/H1N1 infection should be considered.     

Pericardial Effusion and Atrial Thrombosis: A Rare Complication of Childhood Leukemia

Background

Acute lymphoblastic leukemia (ALL) is the most common malignancy diagnosed in children. Extramedullary leukemia including hepatosplenomegaly, lymphadenopathy, and mediastinal mass are the clinical presentations of T-cell ALL. Pericardial effusion and atrial mass are rare presentations of pediatric leukemia.

Case Presentation

We report a 15-year-old girl with T-cell ALL, who presented initially with pericardial effusion and cardiac tamponade and subsequently developed right atrial mass. The mediastinal and right atrial masses were excised by surgical procedure as a treatment strategy for prevention of arrhythmia and sudden death.

Conclusion

T-cell ALL can present with pericardial and mediastinal involvement as an initial manifestation. Pericardiocenthesis and systemic anticancer chemotherapy without local chemotherapy should be considered in these cases.     

Clinical Features of Severe Influenza A (H1N1) Virus Infection

Objective

To highlight the clinical presentations of influenza A (H1N1) infection, for early diagnosis and recognition by the pediatricians.

Methods

In this retrospective study, the medical records of inpatients with influenza A (H1N1) infection between November 1, 2009 and May 31, 2011were reviewed.

Results

Eighty pediatric in-patients with median age 41.9 mo were studied. ARDS (11/80), pneumothorax (8/80), pleural effusion (7/80) and encephalopathy (7/80) were the most frequent complications. Six of 11 ARDS patients died;all of them were under 5 y. The median days of viral shedding was 11.4 d. Slight increase of Il-6, Il-10 and TNF-γ were revealed in some cases.

Conclusions

During late stage of pandemic wave, the majority of patients were young children. Children with severe Influenza A (H1N1) are prone to develop complications, and die from ARDS. If influenza-like illness is accompanied by neurologic signs, influenza A (H1N1) virus infection should be considered. The viral shedding in children is longer than in adults.     

Screening of Pneumococcal Pneumonia by Amplification of Pneumolysin Gene in Children Visiting Hospitals in Lahore,Pakistan

Objective

Streptococcus pneumoniae is a common worldwide potential pathogen causing pneumonia among children and the detection of pneumococcal infections by conventional culturing techniques is cumbersome. The present study describes a comparative analysis of sensitive nested-PCR and bacterial culture in pediatric patients with clinical and radiological indication of S. pneumoniae infection.

Methods

PCR was performed using outer primers to amplify a 348-bp region and inner primers a 208-bp region of the pneumolysin gene. For pneumolysin PCR assay, DNA from peripheral blood and middle ear fluid (MEF) samples was extracted by salting out method. The sensitivity of the assay was evaluated with about 0.06 pg of purified S. pneumoniae genomic DNA.

Findings

Among 90 MEF culture negative samples from acute otitis media pediatric patients, 8.8% pneumolysin-PCR positivity was detected, demonstrating the sensitivity and reliability of PCR for rapid pneumonia evaluation. Binomial test of proportionality performed on (SPSS 17) gives P< 0.05 indicating that PCR technique is statistically significant and sensitive in the diagnosis of S. pneumoniae infection.

Conclusion

The research work evaluated the effectiveness and efficacy of nested-PCR for detecting S. pneumoniae in pediatric patients with clinical and radiological confirmation of bacterial infection. This simplified method permitted quick selection of the patients and played a significant role in preliminary management of pneumococcal infections.     

Estimation of Right Atrial Pressure from the Inspiratory Collapse of the Inferior Vena cava in Pediatric Patients

Objective

Paucity of data exists between mean right atrial pressure (RAP) and inferior vena cava (IVC) size and collapsibility in pediatric patients with congenital heart disease.

Methods

In a prospective study, fifty consecutive pediatric patients with different congenital heart diseases who had right side cardiac catheterization were studied, comparing right atrial pressure with simultaneous M-mode echocardiographic measurement of inferior vena cava diameter. Mean age of the patients was 4.96±4.05 years (30 male and 20 female). Patients were categorized into two groups according to their right atrial pressure (RAP) as measured by cardiac catheterization: Group 1 (40 patients) were those with mean RAP <8 mmHg and group 2 (10 patients) who had a mean RAP≥ 8 mmHg.

Findings

In M-mode echocardiography IVC size was statistically different (P=0.004 and 0.009) in inspiration and expiration in the two groups. Mean RAP was estimated to be>8 mmHg when IVC diameter in inspiration was >3.6 (sensitivity of 100%, specificity of 47.5%, +LR=1.9) or if IVC diameter was >6mm in expiration (sensitivity of 70%, specificity of 87%, +LR=4.67).

Conclusion

This study showed that measurement of IVC size in inspiration and expiration can be used as a reliable method for estimation of mean right atrial pressure.     

Key Ethical Issues in Pediatric Research: Islamic Perspective,Iranian Experience

Objective

The importance of pediatric research especially in the ethically proven trials resulted in considerable legislative attempts in association with compiling ethical guidelines. Because of children''s vulnerability conducting pediatric research raises different ethical issues; the two most important of which are informed consent and risk-benefit assessment. Differences in religious and socio-cultural context limit implication of ethical standards.

Methods

At the aim of finding a solution we critically reviewed guidelines, and literatures as well as Islamic points in addition to comparing different viewpoints in application of ethical standards in pediatric research.

Findings

The literature review showed that pediatric research guidelines and authors’ viewpoints have the same basic ethical core, but there are some variations; depend on cultural, religious, and social differences. Furthermore, these standards have some limitations in defining informed consent according to child''s age and capacity upon application.

Conclusion

In this regard Islamic approach and definition about growth development and puberty sheds light and clarifies a clearer and more rational address to the issue.     

Interventional Electrophysiology in Children: A Single-Center Experience

Objective

Ablation techniques of cardiac arrhythmia in children have significantly progressed in the past decade; however, the number of pediatric ablations is still significantly lower than that in adults. Accordingly, there is less information regarding the success rate and complications in this age group.

Methods

All pediatric ablations conducted between March 2005 and February 2011 at Rajaie Heart Center were studied. Abolishing the arrhythmia source by the end of procedure was considered as success. Recurrences before hospital discharge and those thereafter were named early recurrence and late recurrence, respectively.

Findings

A total of 125 catheter ablations were performed for 112 patients. Of them 118 (94.4%) procedures were successful. The success rate was significantly higher in the patients with atrioventricular nodal reentry tachycardia (AVNRT). Of 105 patients who continued follow-up program, 7 (6.7%) cases experienced recurrence; the recurrence rate was inversely dependent on the patients’ body size (P-value <0.05). There was no mortality. Five cases were complicated during or early after the procedure, all the complications were cured completely.

Conclusion

Therapeutic electrophysiology in children is an effective and relatively low-risk method. The recurrence and complication rates are similar to those reported in adults. Considering our results and the previous reports, pediatric patients with serious arrhythmia should not be deprived from ablation and should not be exposed to long-term toxic drugs.     

Correlation between QRS Duration,Pulmonary Insufficiency and Right Ventricle Performance in Totally Corrected Tetralogy of Fallot

Objective

Despite progresses in surgical correction of Tetralogy of Fallot, pulmonary insufficiency and progressive dysfunction of the right ventricle impress its long-term prognosis. In this study we examined the correlations between QRS duration, pulmonary insufficiency and right ventricular performance index.

Methods

We enrolled 57 repaired Tetralogy of Fallot patients. QRS duration on electrocardiogram, pulmonary regurgitation index (regurgitation time to diastolic time ratio), and right ventricular myocardial performance index were measured.

Findings

There was a strong inverse correlation between QRS duration and pulmonary regurgitation index. However, significant correlation did not exist between QRS duration and right ventricular myocardial performance index. QRS duration ≥160 ms predicted severe pulmonary regurgitation with 100% sensitivity and 87% specificity.

Conclusion

Increased QRS duration can predict severity of pulmonary regurgitation.     

Attempt to Suicide in Young Ages with Epilepsy

Objective

The suicidal rate among epileptic patients is up to 5 times more than general population and depression is the most common mood disturbance among them while usually under noticed. This study was performed to determine possible symptoms of psychiatric disorders for suicidal behavior in pediatric patients with epilepsy that had attempted suicide.

Methods

Among medical records of 1169 patients under 17 years of age being admitted to the exclusive university hospital for poisoning in Tehran since April 2006 to Feb 2008 due to attempt to suicide, 31 cases (13 male and 18 female) with mean age 15.8±1.3 years had concomitant epilepsy. Mental status and epilepsy data sheet of these patients were analyzed.

Findings

The psychological evaluation of these 31 cases revealed long lasting symptoms of psychological disorder in 21 (91.4%) cases before their attempt to suicide while only 5(16%) cases had been noticed for psychiatric care and merely 3 of them had been under treatment.

Conclusion

Despite strong correlation between affective disorders and epilepsy it is unfortunately under-diagnosed or undertreated. Since overdose of antiepileptic drugs used for treatment of epilepsy may be lethal, dismissing suicidal behavior can be life threatening.     

Detecting Common CFTR Mutations by Reverse Dot Blot Hybridization Method in Cystic Fibrosis First Report from Northern Iran

Objective

Cystic fibrosis and its distribution vary widely in different countries and/or ethnic groups. Common cystic fibrosis transmembrane conductance regulator (CFTR) mutations were reported from Iran, but the northern population was not or underrepresented in those studies. The aim of this study was to determine the frequency of common CFTR mutations in children from northern Iran.

Methods

Thirty unrelated Iranian cystic fibrosis patients aged less than 11 years and living in Mazandaran province (in Iran) were screened for 5 common CFTR gene mutations. deltaF508, N1303K, G542X, R347H and W1282X using Reverse Dot Blot method.

Findings

Only one mutation, DeltaF508, was found in 7 patients accounting for 21.7% (13/60) of alleles.

Conclusion

These findings can be used for planning future screening and appropriate genetic counseling programs in Iranian CF families.     

Causes and Types of Precocious Puberty in North-West Iran

Objective

Precocious puberty is of concern because of the underlying disorders, the short adult stature, and the psychosocial difficulties. This study was carried out in order to evaluate the characteristics of children referred to pediatric endocrinology clinic with diagnosis of precocious puberty.

Methods

In a cross-sectional study between February 2007 and September 2009, all of the children referred to pediatric endocrinology clinic in North-West Iran with diagnosis of precocious puberty were recruited.

Findings

Data of 106 girls (82.2%) and 23 boys (17.8%) were analyzed. Mean age of the patients at the time of referral was 6.6±2.8 years (ranging 0.3-14 yr), which was 7±3.9 (ranging 0.3-14 yr) for boys and 6.6±2.5 (ranging 0.8-12 yr) for girls (P=0.6). Out of 129 subjects, 56(43.4%) had precocious puberty, 71.4% (35 cases) of them were due to central precocious puberty and 28.6% (16 cases) were pseudo-precocious puberty. 73 out of 129 subjects (56.6%) were due to normal variants of puberty, normal puberty, and no puberty. 87.5% of subjects with central precocious puberty were idiopathic.

Conclusion

Most of children referred with diagnosis of precocious puberty have benign normal variants. Most of cases with precocious puberty are affected with central precocious puberty, especially with idiopathic form of it.     

Alpha 1 Antitrypsin Deficiency in Infants with Neonatal Cholestasis

Objective

Alpha1-antitrypsin deficiency (A1ATD) is the most important indication for liver transplantation in children. The gene frequencies vary in different ethnic groups. In the present study, we attempt to determine the frequencies of the most common defective alleles, Z and S, in Iranian children suffering from idiopathic neonatal cholestasis. Eighty-seven infants were typed for Z and S alleles.

Methods

In a single center study, 87 consecutive liver biopsies from infants with cholestasis were reviewed and patients with neonatal cholestasis enrolled in the study and cases with confirmed biliary tract atresia excluded. Formalin fixed paraffin embedded blocks were used for DNA extraction. AAT genotype was determined by polymerase chain reaction (PCR) assay and amplification of the two most common deficiency variants, S and Z alleles, and then sequencing of PCR products.

Findings

There were 48 (55.2%) males and 39 (44.8%) females, with a median age of 60 days. Out of 87 of the study subject, 2 (2.2%) were heterozygous for the S allele, and no ZZ, SS or MZ individual was found in the patients. No other polymorphism was found in the sequencing results.

Conclusion

In comparison to other populations, AAT deficiency seems not to be an important etiologic factor for neonatal cholestatic liver disease in Iran; however, further studies are recommended to estimate the true mutant gene frequencies.     

New Single Nucleotide Deletion In the SMPD1 Gene Causes Niemann Pick Disease Type A in a Child from Southwest Iran: A Case Report

Objective

Niemann Pick disease (NPD) type A (NPA: MIM #257200) is a lipid storage disorder with an autosomal recessive inheritance and occurrs by defect of the SMPD1 gene encoding sphingomyelinase. Disruption of this enzyme leads to the accumulation of sphingomyelin in brain and liver, which in turn causes dysfunction or damage of tissue.

Methods

We report firstly a 2.5 year old boy with NPA in southwest Iran. Initially, the diagnosis was resulted on the basis of clinical symptoms. The genomic DNA of the suspected individual was subjected to exon sequencing of the SMPD1 gene. According to the human reference sequence {"type":"entrez-nucleotide","attrs":{"text":"NM_000543.4","term_id":"300795568","term_text":"NM_000543.4"}}NM_000543.4, a novel single guanine deletion resulting in a frameshift mutation (p.Gly247Alafs*9) was observed in the SMPD1 gene that might be causative for the outcome of the disease.

Findings

The present report is the first molecular genetics diagnosis of the NPA in southwest Iran. The detected deletion in the SMPD1 gene is remarkable because of its novelty.

Conclusion

Despite similar morbidity SGA infants exhibited higher lethal complication rates following delayed meconium passage compared to AGA infants.     

Predictors of Mortality in Hospitalized Children with Pandemic H1N1 Influenza 2009 in Pune, India

Objective

To analyse the factors associated with increased mortality among Indian Children with H1N1.

Methods

Data were abstracted from available hospital records of children less than 12?y of age, who were admitted to Sassoon General Hospital in Pune, India, with confirmed pandemic 2009 H1N1 influenza infection from August 2009 through January 2010. Logistic regression analysis was used to identify clinical characteristics associated with mortality.

Results

Of 775 pediatric cases admitted with Influenza Like Illness (ILI), 92 (11.8%) had confirmed H1N1 influenza infection. The median age of HIN1 cases was 2.5?y; 13 (14%) had an associated co-morbid condition. Median duration of symptoms was 4?d (interquartile range (IQR), 3?C7?d). All 92 H1N1 cases received oseltamivir and empiric antimicrobials on admission. Intensive care unit (ICU) admission was required for 88 (96%) children, and 20 (23%) required mechanical ventilation.Fifteen children (16%) died; mortality was associated with presence of diffuse alveolar infiltrate on admission chest radiography (odds ratio (OR) 45, 95%CI :5.4?C370; p?p?=?0.001), SpO₂ <80% on admission (OR 32.8, 95% CI: 5.8?C185.5; p?p?n?=?4 each, 27%) with gram positive organisms consistent with severe viral and bacterial co-infection.

Conclusions

Hypoxia, ARDS and use of corticosteroids in children with ARDS who were mechanically ventilated were the factors associated with increased odds of mortality. Necropsy also suggested bacterial co-infection as a risk factor.     

Childhood Pityriasis rosea inversa without Herald Patch Mimicking Cutaneous Mastocytosis

Background

Pityriasis rosea is a self-limited inflammatory condition of the skin that mostly affects young adults. Several less common atypical presentations have been reported.

Case Presentation

A 6-year old girl with red-brown maculopapular eruption sized 0, 5–1 cm in diameter localized on neck, trunk and popliteal region visited our general pediatric outpatient clinic. The eruption was wide spread especially on flexural areas. After consulting dermatologist skin biopsy was performed. According to clinical and histopathological findings as inverse (flexural) pityriasis rosea was diagnosed. For treatment, systemic antihistamine, topical corticosteroid cream and emollient were applied. The lesions healed in two months. Spontaneous healing of the eruption also confirmed the diagnosis of pityriasis rosea.

Conclusion

We present this interesting pediatric case to show and discuss pityriasis rosea, atypical presentations, differential diagnosis and the importance of dermatological examination and importance of dermatologic consultation for pediatric patients with skin eruption.     

Association between Helicobacter pylori,cagA, and vacA Status and Clinical Presentation in Iranian Children

Objective

Seroprevalence of H. pylori infection in Iran exceeds 65% of pediatric population. In this study, we intended to find association between the virulence genes (cagA and vacA) and clinical presentations.

Methods

H. pylori isolates were achieved from the gastric mucosa of children. In each case, the gastric biopsy specimens were cultured and the organisms identified. Detection of different genotypes was carried out by PCR method.

Findings

A total of 106 biopsy specimens were cultured and 33 H. pylori isolates obtained. Among these 33 H. pylori strains 24 (73%) were cagA-positive. Genotypes of vacA s1m2, s1m1, s2m2, and s2m1 were 45.5%, 30.3%, 21.2%, and 3%, respectively. Most female patients were infected with genotype s1m2. The vacA-m1 strains were significantly more common in patients with nodular gastritis. There were no statistical differences between the vacA and cagA genotypes and clinical outcomes.

Conclusion

The frequency of cagA genotype was high. In this study, nodular gastritis was a common finding and was rather significantly associated with m1 allele of vacA.     

Pediatric Ureteroceles: Diagnosis,Management and Treatment Options

Objective

The aim of the study was to evaluate clinical characteristics of ureteroceles particularly for diagnostic and treatment challenges.

Methods

Data about patients treated for ureterocele in the two hospital clinics during 1996- 2009 are retrospectively evaluated.

Findings

There were 12 girls and 7 boys. Symptomatic urinary tract infection was found in twelve cases. Ureterocele was associated with duplex systems in eleven cases. Vesicoureteral reflux was detected in 4 patients. Bladder diverticulum complicated with ureterocele in 1 patient. Ultrasonography diagnosed ureterocele in 12 patients. Renal scarring was detected in 6 patients at the side of ureterocele. Fifteen patients showed varying degrees of hydro-ureteronephrosis. Surgical therapy included upper pole nephrectomy in 3 cases. Bladder level reconstruction was performed in 11 cases. Five patients were treated only by endoscopic incision. In the follow up period 4 patients showed long term urinary tract infections whereas 3 of them were treated endoscopically. Postoperative reflux was still present in two patients who were treated by endoscopic incision.

Conclusion

Ureterocele diagnosis and treatment show challenges. Urinary tract infection is important marker for urinary system evaluation. Preoperative management generally depends on a combination of diagnostic methods. Endoscopic incision needs serious follow up for postoperative problems.     

Laparoscopic upper pole heminephroureterectomy in children: Seven-year experience

Background:

Minimally invasive surgery is the current approach to perform heminephroureterectomy (HN) in children. This can be obtained through a transperitoneal (TP) or a retroperitoneal approach. Here, we report our experience using a TP approach.

Materials and Methods:

From 2005 to 2014, 22 TP laparoscopic upper poles HN were performed at our institution. There were nine girls and 13 boys aged between 20 months and 6 years (mean age 3.9). Eight patients were diagnosed prenatally, 17 patients presented with urinary tract infection (UTI) and three with vomiting and failure to thrive. The indication for HN was reflux nephropathy and UTI in non-functioning upper pole in 19 patients and cystic dysplasia in 1 patient. The surgical technique involved the following steps: Cystoscopic recognition; positioning of 3-4 trocar (right HN); identification of the kidney (detachment of the colon); isolation and low ligation of the dilated ureter; decrossing from renal vessels; section of the parenchyma by LigaSure; haemostasis with clips and LigaSure; drain.

Results:

The mean operative time was 154 min (range: 81-220 min). All patients were discharged from the 2^nd to 4^th day. Neither major complication nor conversion was recorded. 1 patient presented leakage of urine for 7 days from the drainage which resolved spontaneously. At ultrasound follow-up, 5 patients showed a secondary perirenal cyst, 2-5 cm diameter that resolved spontaneously.

Conclusion:

The results indicate that laparoscopic upper pole heminephrectomy is the treatment of choice in cases of non-functioning dilated lower segments of duplicated kidneys. The use of laparoscopic approach offers a good working space, a good visual control of the vessels and allows a very low isolation of the ureteral stump which counterbalance the peritoneal violation.Key words: Duplex kidney, heminephrectomy, infants, laparoscopy