Nasopharyngeal structure development in patients with cleft palate who underwent repair surgery.

OBJECTIVE: This study aimed to compare the developmental characteristics of bony nasopharyngeal (NP) in children with cleft palate (CP) and non-cleft subjects. METHODS: This study was conducted on 64 non-syndromic CP patients who underwent repair surgery and 23 non-cleft subjects as controls. Lateral cephalograms were performed on participants at rest in the natural head position. The X-coordinate and the Y-coordinate of three points on cephalograms were determined as Hormion (Ho), posterior maxillary point (PMP) and anterior point of the atlas (At) representing the anterior-posterior (AP) and vertical dimension of the NP. The linear dimension of the NP (i.e. Ho-At, Ho-PMP, At-PMP) and its area was also calculated. RESULTS: The bony structures of NP in the clef-affected subjects in isolated CP subgroup, had a significantly greater downward development in the maxillary region (PMP) (both with p = 0.001), more linear growth in cranial-maxillary (Ho-PMP) dimension (p = 0.017 and 0.004, respectively), and larger area (p = 0.017 and <0.001, respectively), when compared to normal subjects. There was no significant difference between either the unilateral cleft lip and palate (CLP) or bilateral CLP group with the control group regarding AP, vertical, and linear growth of the NP and its area (P > 0.05). CONCLUSIONS: Patients with repaired CP had downward deviated posterior maxilla, more linear growth in cranial-maxillary dimension and larger area compared to normal subjects. Among CP subtypes, a balanced growth was observed among repaired UCLP and BCLP patients, suggesting that with appropriate repaired surgery, normal development of the NP region could be expected in these subtypes.

Clinical and Demographical Characteristics of Cleft Lip and/or Palate in the Northwest of Iran: An Analysis of 1500 Patients.

OBJECTIVE: Orofacial clefts (OFCs) can occur as an isolated defect or as a manifestation of other syndromes. The current study aimed to evaluate demographic characteristics and distribution of different types of accompanying anomalies for OFCs in the northwest of Iran. DESIGN: A retrospective cohort study. SETTING: Tertiary pediatric hospital. PATIENTS AND PARTICIPANTS: This study was conducted on 1500 cleft lip and/or palate patients born between July 2010 and June 2020 in the northwest of Iran. MAIN OUTCOME MEASURES: Demographic and clinical characteristics of the children with OFCs including familial history, accompanying anomalies and syndromes, maternal passive smoking, mothers' and fathers' age, consanguineous marriage, and birth order. RESULTS: Among 1500 patients, 441 had cleft lip, 615 had cleft palate, and 444 had cleft lip and palate. The positive family history of OFCs was found to be 20.9% to 25.4% depending on the cleft type. Accompanying anomalies were identified in 29.8% of cases. Cardiac, facial, and ear abnormalities were the most common types. Also, 2.9% were identified with syndromes and sequences. These included Pierre Robin Sequence, Velo-cardio-facial syndrome, and Down syndrome most frequently. CONCLUSION: These findings may provide references for appropriate resources to establish and direct counseling and primary preventive projects in the northwest of Iran.

Comparison of two-flap palatoplasty plus intravelar veloplasty technique with and without double-layer Z-plasty on the soft palate length in children with cleft palate.

PURPOSE: Velopharyngeal failure occurs as a result of a nasopharyngeal gap following inadequate velopharyngeal closure for structural or functional cause. We aimed to compare the soft palate length in two-flap palatoplasty with intravelar veloplasty (IVV) and two-flap palatoplasty with IVV plus double-layer Z-plasty combination in patients with cleft palate. METHODS: This clinical trial was conducted on infants aged 9 to 12 months with cleft palate in two groups. The method of two-flap palatoplasty with IVV technique and two-flap palatoplasty with IVV plus double-layer Z-plasty was compared in terms of soft palate length which was measured during operation and short-term complications. RESULTS: This study was conducted on 62 infants including 30 patients in two-flap palatoplasty with IVV group and 32 patients in two-flap palatoplasty with IVV plus double-layer Z-plasty group. The soft palate length in two-flap palatoplasty and IVV with and without Z-plasty groups before surgery was 17.56 ± 2.05 and 17.68 ± 1.88 mm, respectively (P = 0.561). After surgery, the soft palate length was significantly higher in two-flap palatoplasty with IVV plus Z-plasty group (22.43 ± 2.73 mm vs. 20.56 ± 2.42 mm) (P = 0.032). CONCLUSION: The two-flap palatoplasty with IVV plus Z-plasty technique is a suitable method for increasing the palatal length in infants with cleft palate. On the other hand, the addition of Z-plasty method could increase the length of the palate. Moreover, the complications are very low and further trials for development of this method on patients with cleft palate are recommended. TRIAL REGISTRATION NUMBER (TRN): IRCT2017032423559N11.

Occult HCV and occult HBV coinfection in Iranian human immunodeficiency virus-infected individuals.

The presence of hepatitis C virus (HCV) genome in liver biopsy or peripheral blood mononuclear cell (PBMC) specimens in the absence of detectable HCV-RNA in plasma of the people with or without anti-HCV antibodies has defined as occult HCV infection (OCI), whereas occult hepatitis B virus infection (OBI) is detection of hepatitis B virus (HBV) genome in the absence of traceable hepatitis B surface antigen in the plasma samples of patients. The purpose of this study is to determine the presence of OBI and OCI in human immunodeficiency virus (HIV)-infected individuals. In this cross-sectional research, 190 Iranian HIV-infected individuals were enrolled from September 2015 to February 2019. All participants were tested regarding various serological markers for HCV and HBV infections. Viral RNA and DNA were extracted from plasma and PBMC specimens, and the presence of HCV-RNA in plasma and PBMC samples was tested using reverse transcriptase-nested polymerase chain reaction (PCR), HBV viral load was determined in plasma samples using COBAS TaqMan 48 Kit, and also the presence of the HBV-DNA in PBMC samples was tested by real-time PCR. In this study, the prevalence of OBI and OCI in HIV-infected individuals was 3.1% and 11.4%, respectively. The genotypes of HCV in the patients with OCI were as follows: 57.1% were infected with subtype 3a, 35.7% were infected with subtype 1a, and 7.1% was infected with subtype 1b. It is noteworthy that in this study, two patients (1.1%) had OCI/OBI coinfections. The present study revealed that 1.1% of Iranian HIV-infected individuals had OBI and OCI at the same time. Therefore, it seems that designing prospective surveys to determine the presence of this coinfection in HIV-infected individuals is informative.

Effects of phenytoin spray in prevention of fistula formation following cleft palate repair.

BACKGROUND: The effectiveness of topical phenytoin has been reported for the treatment of oral biopsy ulcers, chemotherapy-induced oral mucositis, and chronic periodontitis. This study aimed to investigate the effects of topical phenytoin 2% on the prevention of fistula formation after cleft palate repair. METHOD: This randomized clinical trial studied patients with nonsyndromic cleft palate who were referred to a tertiary center and underwent cleft palate repair from March 2010 to February 2015. Patients in the phenytoin group received phenytoin spray 2% for 8 weeks and were compared with an age- and sex-matched control group. RESULTS: A total of 160 patients in two phenytoin and control groups (n = 80 for each group) were recruited to the study. The mean ages of patients in the phenytoin and control groups were 11.42 ± 1.30 and 11.08 ± 1.25 months, respectively. The results showed that six patients (7.5%) in the phenytoin group and 15 patients (18.8%) in the control group formed fistulas during the 6-month follow-up period. There was a significant difference in fistula formation between the phenytoin and control groups (p = 0.035). Furthermore, fistula size was significantly smaller in the phenytoin group compared with the control group (p < 0.001). CONCLUSION: More frequent use of phenytoin spray can be considered, although there is insufficient information on the long-term side-effects of the chosen drug.

Prevalence of occult hepatitis C virus infection in the Iranian patients with human immunodeficiency virus infection.

Occult hepatitis C virus (HCV) infection is a new form of chronic HCV infection described by the presence of the genomic HCV-RNA in liver biopsy and/or peripheral blood mononuclear cell (PBMC) samples, and undetectable levels or absence of HCV-RNA and in the absence or presence of anti HCV antibodies in the plasma specimens. The aim of the present study was to evaluate the occurrence of occult HCV infection (OCI) among Iranian subjects infected with human immunodeficiency virus (HIV) using RT-nested PCR. From March 2014 until April 2015, 109 Iranian patients with established HIV infection were enrolled in this cross-sectional study. After extraction of viral RNA from the plasma and PBMC samples, HCV-RNA status was examined by RT-nested PCR using primers from the 5'-NTR. HCV genotyping was conducted using RFLP analysis. For the confirmation of HCV genotyping by RFLP method, the PCR products were sequenced. Of the 109 patients, 50 were positive for antibodies against HCV. The HCV-RNA was detected in PBMC specimens in 6 (10.2%) out of the total 59 patients negative for anti-HCV Abs and undetectable plasma HCV-RNA and also from 4 (8.0%) out of the total 50 patients positive for anti-HCV Abs and undetectable plasma HCV-RNA. HCV genotyping analysis showed that 6 (60.0%) patients were infected with HCV subtype 3a, 3 (30.0%) were infected with HCV subtype 1a and 1 (10.0%) patient was infected with HCV subtype 1b. This study revealed the incidence of OCI (9.2%) in HIV-infected Iranian patients. Hence, designing prospective studies focusing on the detection of OCI in these patients would provide more information. J. Med. Virol. 88:1960-1966, 2016. © 2016 Wiley Periodicals, Inc.

Prevalence of Associated Anomalies in Cleft Lip and/or Palate Patients.

INTRODUCTION: Orofacial clefts are among the most common congenital anomalies. Patients presenting with orofacial clefts often require surgery or other complex procedures. A cleft lip or palate can be a single anomaly or a part of multiple congenital anomalies. The reported prevalence of cleft disease and associated anomalies varies widely across the literature, and is dependent on the diagnostic procedure used. In this study we determined the prevalence of associated anomalies in patients with a cleft lip and/or palate, with a specific focus on cardiac anomalies. MATERIALS AND METHODS: In this cross-sectional study, 526 patients with a cleft lip and /or palate admitted to the children's referral hospital between 2006 and 2011 were evaluated. All associated anomalies were detected and recorded. Patient information collected included age, gender, type and side of cleft, craniofacial anomalies and presence of other anomalies, including cardiac anomalies. Data were analyzed using SPSS version 16. RESULTS: Of the 526 patients enrolled in the study, 58% (305) were male and 42% (221) were female. In total, 75% of patients (396) were aged between 4 and 8 years and 25% (130) were aged less than 4 years. The most common cleft type in our study was bilateral cleft palate. The most commonly associated anomaly among cleft patients, in 12% of cleft patients, was a cardiac anomaly. The most common cardiac anomaly was atrial septal defect (ASD). CONCLUSION: The prevalence of associated anomalies among orofacial cleft patients is high. The most common associated anomaly is cardiac anomaly, with ASD being the most common cardiac anomaly. There are no significant relationships between type of cleft and associated cardiac anomalies.

The Association of Substitutions in the Hepatitis C Virus Subtype 1b Core Gene and IL28B Polymorphisms With the Response to Peg-IFNα-2a/RBV Combination Therapy in Azerbaijani Patients.

BACKGROUND: The hepatitis C virus (HCV) infection has been identified as a leading cause of progressive liver diseases worldwide. Despite new treatment strategies, pegylated interferon alfa-2a (Peg-IFNα-2a), in combination with ribavirin (RBV), still represents the gold standard of therapy for hepatitis C in developing countries. OBJECTIVES: The aim of this study was to investigate the association of substitutions in the HCV subtype 1b (HCV-1b) core protein and the rs12979860 polymorphism in the interleukin 28B gene (IL28B) with the response to Peg-IFNα-2a/RBV combination therapy in Azerbaijani patients. PATIENTS AND METHODS: A total of fifty-one chronically HCV-1b-infected Azerbaijani patients were enrolled in this cross-sectional study from March 2010 to June 2015. After RNA extraction from pre-treatment plasma, the core region of the HCV genome was amplified using the nested reverse transcription (RT) polymerase chain reaction (PCR) method, followed by standard sequencing. In addition, genomic DNA was extracted from peripheral blood mononuclear cell (PBMC) specimens, and the rs12979860 single nucleotide polymorphism (SNP) was identified using a PCR-restriction fragment length polymorphism (PCR-RFLP) assay. RESULTS: In this study, a significant association was observed between the non-responders and relapsers to antiviral therapy and substitutions in the HCV-1b core region at positions 43 (R43K, P = 0.047), 70 (R70Q, P < 0.001), 91 (M91L, P = 0.037), and 106 (S106N, P = 0.018). Concerning the IL28B polymorphism, the results showed that sustained virological response was significantly associated with homozygous CC patients (P = 0.009) as compared with other genotypes, while homozygous TT subjects were associated with HCV relapse after therapy (P = 0.006). CONCLUSIONS: The data of the present study suggest that amino acid substitutions at position 43, 70, 91, and 106 in the HCV-1b core protein are correlated with the response to the Peg-IFNα-2a/RBV treatment in Azerbaijani patients with chronic hepatitis C. Moreover, host genetic polymorphisms, such as those of the IL28B locus, might be useful for predicting the responsiveness to Peg-IFNα-2a/RBV combination therapy against HCV.

Prevalence of occult hepatitis C virus infection in Iranian patients with beta thalassemia major.

Occult hepatitis C virus infection (OCI) is a new pathological form of chronic hepatitis virus (HCV) infection characterized by the presence of HCV RNA in liver biopsy and/or peripheral blood mononuclear cell (PBMC) specimens and the absence of HCV RNA and anti-HCV antibodies (Abs) in plasma samples. ß-thalassemia major is a hereditary recessive blood disease with deficiency in the hemoglobin beta chain. Thalassemic patients need blood transfusion therapy; repeated blood transfusion increases the risk of viral blood-borne infection. The aim of this study was to determine the prevalence of OCI in Iranian patients with ß-thalassemia major. From February 2015 to November 2015, a total of 147 Iranian patients with ß-thalassemia major were enrolled in this cross-sectional study. After extraction of viral RNA from the plasma and PBMC samples, HCV genomic RNA in the specimens was amplified by RT-nested PCR using primers from the 5'-UTR. The HCV genotypes of the positive specimens were tested using the RFLP assay. To confirm the HCV genotypes, the 5'-UTR fragment was amplified and cloned into the pJET1.2/blunt cloning vector and then sequenced. Out of 147 patients, 106 (72.1 %) were negative for anti-HCV Abs and HCV RNA. HCV RNA was found in PBMC specimens of six (5.7 %) patients, from a total of 106 patients with undetectable plasma HCV RNA and anti-HCV Abs. Therefore, six out of 106 patients had OCI. HCV genotyping revealed that three patients were infected with HCV subtype 1b, two patients were infected with HCV subtype 3a, and one patient was infected with HCV subtype 1a. These results revealed that Iranian patients with beta-thalassemia major might have OCI. Therefore, it seems that the design of a study to identify this infection in patients with ß-thalassemia major would provide valuable information.

Effects of adenotonsillar hypertrophy corrective surgery on nocturnal enuresis of children.

BACKGROUND: Nocturnal enuresis is considered a common urological complaint especially among children. Respiratory obstructive diseases have been one of the possible etiologies of such a condition. The most common type of upper respiratory obstructive diseases in childhood is adenotonsillar hypertrophy. In this study, it was tried to estimate the prevalence of nocturnal enuresis in adenotonsillar hypertrophy as an upper obstructive airway disease and cure rate after corrective operation. MATERIALS AND METHODS: In this longitudinal study, 184 children with adenotonsillar hypertrophy as case group and 200 healthy children as control group were randomly compared for nocturnal enuresis incidence and risk factors. Then they were followed after 6 months to estimate the cure rate after corrective operation. RESULTS: In case group, nocturnal enuresis was more common than control group (26% vs. 17%, P = 0.1). Factors which had a role in enuresis in case group were family history (P = 0.03) and male sex (P = 0.05). Three months after surgery, 48% of children totally cured from enuresis (P = 0.001) and 71% cured both partially or totally (P = 0.03). The response rate after moderate obstruction relieving was 100% while that in severe cases was 60% (P = 0.2). CONCLUSIONS: Nocturnal enuresis due to upper obstructive airway disease occurs more in male and in the presence of family history. The cure rate after 6 months of operation was more prominent in moderate obstruction which suggests enuresis in severe airway obstruction may need a longer time to subside. Cure rate of primary enuresis due to obstructive airway disease after 6 months of relieving was 48% in children over 5 years old.

Detection of Hepatitis B Virus Covalently Closed Circular DNA in the Plasma of Iranian HBeAg-Negative Patients With Chronic Hepatitis B.

BACKGROUND: Covalently closed circular DNA (cccDNA) of hepatitis B virus (HBV) is a marker of HBV replication in the liver of patients infected with HBV. OBJECTIVES: This study aimed to investigate the association between the presence of cccDNA in the plasma samples of Iranian treatment-naive patients with chronic hepatitis B infection and HBV viral load and HBsAg levels. PATIENTS AND METHODS: From April 2012 to May 2015, 106 treatment-naive patients with chronic hepatitis B infection were enrolled in this cross-sectional study. The HBsAg titer was measured by the Roche HBsAg II assay on the Cobas e411 system, and HBV DNA quantitation was performed using the COBAS TaqMan 48 kit. Real-time polymerase chain reaction was performed for the detection of HBV cccDNA. RESULTS: The mean (SD) age of the patients was 41.1 ± 12.4 years (range, 20 - 62 years). From a total of 106 study participants, 67 (63.2%) were males. The HBV cccDNA was detected in plasma specimens in 19 (17.9%) out of the total 106 patients, and a significant relationship was found between the presence of cccDNA in plasma sample of males (23.9%) and females (7.7%) (P = 0.039). Also, a significant correlation was found between the presence of cccDNA in plasma sample of the patients and HBV viral load level (P < 0.0001) and HBsAg titer (P = 0.0043). CONCLUSIONS: This study showed that cccDNA can be detected in the plasma specimen of 17.9% of Iranian treatment-naive patients with chronic hepatitis B infection. Therefore, designing prospective studies focusing on the detection of cccDNA in these patients would provide more information.

Common Mutations of the Methylenetetrahydrofolate Reductase (MTHFR) Gene in Non-Syndromic Cleft Lips and Palates Children in North-West of Iran.

INTRODUCTION: Cleft lips and cleft palates are common congenital abnormalities in children. Various chromosomal loci have been suggested to be responsible the development of these abnormalities. The present study was carried out to investigate the association between the suspected genes (methylenetetrahydrofolate reductase [MTHFR] A1298C and C677T) that might contribute into the etiology of these disorders through application of molecular methods. MATERIALS AND METHODS: This cross-sectional and explanatory study was carried out on a study population of 65 affected children, 130 respective parents and 50 healthy individuals between 2009 and 2012 at Tabriz University of Medical Sciences, IR Iran. After DNA extraction, amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) and restriction fragment length polymorphism (RFLP)-PCR were used respectively to investigate the C677T and A1298C mutations for the MTHFR gene. RESULTS: There was a significant difference in the rates of the C677T mutation when affected patients and their fathers were compared with the control group (odds ratio [OR]=0.44) (OR=0.64). However, there was no significant difference observed in the rate of this mutation between the patients' mothers and the control group (OR=1.35). In addition, the abnormality rate was higher in patients with the A1298C mutation and their parents, when compared with the control group. This abnormality rate was higher for the affected children and their fathers in comparison with their mothers (Fathers, OR=0.26; Mothers, OR=0.65; Children, OR=0.55). No significant difference was seen in the rate of the polymorphism C677T in its CC, when the affected children and their parents were compared with the control group. However, there was a significant difference in the A1298C mutation. CONCLUSION: An association was seen between the A1298C mutation and cleft lip and cleft palate abnormalities in Iran. However, there seems to be a stronger relationship between the C67TT mutation and these abnormalities in other countries, which could be explained by racial differences. Moreover, this association was more notable between the affected children and their fathers than their mothers. The findings in this study may be helpful in future studies and screening programs.

Medical treatment of allergy in children with recurrent or chronic sinusitis.

BACKGROUND: Diagnosis and treatment of chronic and recurrent sinusitis in children is of potential importance and many factors can havean influence on it. This study assessed the chronic and recurrent sinusitis and affecting factors, as well as the role of allergy in its course and treatment in children. MATERIALS AND METHODS: A total of 106 children with the diagnosis of chronic or recurrent sinusitis, referred to specialty clinics of otolaryngology and allergy of Tabriz Children Educational-Medical centres since 2010 to 2012, were enrolled. The history and physicalexamination findings were recorded for all patients and allergy Prick test was done for all. Response to treatment was evaluated during the follow-up visits. RESULTS: The mean age of studied patients was 6.5 ± 2.9 years. Of all the patients, 54 (50.9%) were male and 52 (49.1%) were female. Skin Prick test was positive in 69.8%. Response to treatment was seen in 86.8% of the patients while 7.5% did not have any favourable outcome. Anti-allergic treatment caused better outcomes in patients with positive Skin Prick test than those with negative results. CONCLUSION: The prevalence of allergic disease in children with chronic or recurrent sinusitis is considerable and anti-allergic treatments can result infavourable therapeutic outcomes in children with sinusitis, especially with positive skin prick test results.

First branchial cleft fistula associated with external auditory canal stenosis and middle ear cholesteatoma.

INTRODUCTION: First branchial cleft anomalies manifest with duplication of the external auditory canal. CASE REPORT: This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection. CONCLUSION: It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear.

Distribution of hepatitis B virus genotypes in azerbaijani patients with chronic hepatitis B infection.

BACKGROUND: Hepatitis B virus (HBV) has been classified into ten genotypes (A-J) based on genome sequence divergence, which is very important for etiological and clinical investigations. HBV genotypes have distinct geographical distributions worldwide. OBJECTIVES: The aim of this study was to investigate the distribution of HBV genotypes among Azerbaijani patients with chronic hepatitis B, came from the Republic of Azerbaijan country to Iran to receive medical care. PATIENTS AND METHODS: One hundred and three patients with chronic HBV infection, referred to hospitals related to Iran University of Medical Sciences and Tehran Hepatitis Center from August 2011 to July 2014, were enrolled in this cross sectional study. About 3-milliliter of peripheral blood was taken from each patient. After viral DNA extraction, HBV genotypes were tested using the INNO-LiPA™ HBV kit (Innogenetics, Ghent, Belgium). HBV genotyping was confirmed using sequencing of hepatitis B surface antigen (HBsAg) and polymerase (pol) regions of HBV. RESULTS: The mean age of patients was 35.9 ± 11.7 years (19-66). Of 103 patients, 72 (69.9%) were male. In the present study, the predominant HBV genotype was D (93.2%) followed by genotype A (5.8%) and concurrent infection with A and D genotypes (0.97%). CONCLUSIONS: The main and frequent HBV genotype among Azerbaijani patients with chronic hepatitis B virus infection was genotype D followed by genotype A.

Effect of peripheral edema on oscillometric blood pressure measurement.

INTRODUCTION: Blood pressure (BP) measurement is essential for epidemiological studies and clinical decisions. It seems that tissue characteristics can affect BP results and we try to find edema effect on BP results taken by different methods. METHODS: BP of 55 children before open heart surgery were measured and compared according to three methods: Arterial as standard and reference, oscillometric and auscultatory methods. Peripheral edema as a tissue characteristic was defined in higher than +2 as marked edema and in equal or lower than +2 as no edema. STATISTICAL ANALYSES: data was expressed as Mean and 95% of confidence interval (CI 95%). Comparison of two groups was performed by T independent test and of more than two groups by ANOVA test. Mann-Whitney U and paired T-test were used for serially comparisons of changes. P less than 0.05 was considered significant. RESULTS: Fifty five children aged 29.4±3.9 months were divided into two groups: 10 children with peripheral edema beyond +2 and 45 cases without edema. Oscillometric method overestimated systolic BP and the Mean (CI 95%) difference of oscillometric to arterial was 4.8 (8/-1, P=0.02) in edematous and 4.2 (7/1, p=0.004) in non edematous. Oscillometric method underestimated diastolic BP as -9 (-1.8/-16.5, P=0.03) in edematous group and 2.6 (-0.7/+5, P= 0.2) in non edematous compared to arterial method. CONCLUSION: Oscillometric device standards cannot cover all specific clinical conditions. It underestimates diastolic BP significantly in edematous children, which was 9.2 mmHg in average beyond the acceptable standards.

Distribution of hepatitis C virus genotypes among azerbaijani patients in capital city of iran-tehran.

BACKGROUND: Determination of the Hepatitis C virus (HCV) genotype distributed in a particular area has an important role on public health throughout the world. OBJECTIVES: The aim of this study was to determine the frequency of HCV genotypes in Azerbaijani patients. PATIENTS AND METHODS: From March 2010 until March 2012, 235 Azerbaijani patients with established chronic hepatitis C, referred to Hospitals related to Iran University of Medical Sciences and Tehran Hepatitis Center, Clinical department of Baqiyatallah Research Center for Gastroeneterology and Liver Disease, were enrolled in this cross sectional study. About 5 mL of peripheral blood was collected from patients and after separation of plasma, viral RNA extracted. HCV-RNA were amplified by RT-nested PCR using primers from the 5´-UTR and genotyped by RFLP assay, and then HCV genotypes were confirmed using sequencing of cloned PCR products into pJET1.2/blunt cloning vector. RESULTS: HCV genotyping of positive plasma samples demonstrated that predominant HCV subtype was noted for 1b (71.1%) followed by subtype 3a (17.0%), genotype 2 (6.8%), 1a (1.7%), and mixed infection (3.4%). The mean ± SD age of patients was 37.3 ± 11.8 (range: 2-63) years. Out of 235 patients, 139 (59.1%) were male. The frequency of HCV subtype 3a was higher in patients under 40 years old (3a: 18.1% vs. 15.0%), and subtype 3a was higher in male patients (3a: 18.7% vs. 14.6%). CONCLUSIONS: The current study shows that the predominant HCV genotype among Azerbaijani patients with established chronic hepatitis C is subtype 1b (71.1%) followed by subtype 3a (17.0%).

Occult hepatitis C virus infection in candidates for liver transplant with cryptogenic cirrhosis.

BACKGROUND: Occult hepatitis C virus (HCV) infection is a new entity described by the presence of HCV-RNA in liver biopsy and/or peripheral blood mononuclear cell (PBMC) specimens, and undetectable levels or absence of HCV-RNA and in the absence or presence of anti HCV antibodies in plasma by current laboratory methods. OBJECTIVES: To evaluate the detection of HCV-RNA in PBMC specimens of the liver transplant candidates with cryptogenic cirrhosis by reverse transcriptase-nested polymerase chain reaction (RT-nested PCR). PATIENTS AND METHODS: From November 2007 to March 2013, 45 patients from Liver Transplant Center of Imam Khomeini Hospital, Tehran, were enrolled in this cross sectional study. PBMC specimens were separated from the peripheral blood sample. After extraction of RNA from plasma and PBMC specimens, HCV-RNA status was tested by RT-nested PCR. The 5'-untranslated region (5'-UTR) genotyping of HCV-RNA amplified from PBMC specimens was performed by a standard methodology with the INNO-LiPA(TM) HCV II kit. The PCR products of 5'-UTR were sequenced after cloning into the pJET1.2 / blunt cloning vector. RESULTS: Of 45 patients, 4 (8.9% [95% CI: 4.4-15.6]) had detectable genomic HCV-RNA in their PBMC specimens. HCV genotypes were determined in the PBMCs of these subjects showed that 2 (50.0%) subjects with occult HCV infection had HCV subtype 3a, and 2 (50.0%) had HCV subtype 1b. CONCLUSIONS: This study found that 8.9 % of the Iranian candidates for liver transplant with cryptogenic cirrhosis had occult HCV infection. Therefore, designing prospective studies focusing on the diagnosis of occult HCV infection in these subjects prior to liver transplantation could be valuable.

Distribution of hepatitis C virus genotypes in Iranian chronic infected patients.

BACKGROUND: Hepatitis C virus (HCV) has different genotypes throughout the world. Since the determination of which antiviral treatment to be applied is related to HCV genotypes, identification of an individual's HCV genotypes prior to antiviral therapy is critical. OBJECTIVES: The purpose of this study was to investigate the distribution of HCV genotypes in a large population of Iranian HCV infected patients. PATIENTS AND METHODS: Eleven thousand, five hundred and sixty one patients with chronic HCV infection which referred to hospitals related to the Tehran University of Medical Sciences and Tehran Hepatitis Center-Clinical Department of Baqiyatallah Research Center for Gastroeneterology and Liver Disease from March 2003 to December 2011 were enrolled. Following extraction of viral RNA of the serum, HCV-RNA was detected using reverse transcriptase-nested polymerase chain reaction (RT-nested PCR) and then HCV genotypes analyzed by restriction fragment length polymorphism (RFLP) assay. RESULTS: The mean age of patients was 37.6 ± 14.2 years (range: 1-87). The highest frequency was noted for subtype 1a (44.9%) followed by subtype 3a (39.6%), and 1b (11.3%). Mixed HCV genotypes were also found in 2.5% of the total cases. Subtype 1a was the most frequent genotype in patients over 40 years of age (46.1% versus 42.4%) and subtype 3a was the most frequent in patients under 40 years old (41.5% versus 38.9%). CONCLUSIONS: This study suggested that the dominant HCV subtype among Iranian patients was 1a followed by subtype 3a.

A case report: nager acrofacial dysostosis.

INTRODUCTION: Nager syndrome is a malformation resulting from problems in the development of the first and second branchial arches and limb buds. The cause of the abnormal development of the pharyngeal arches in Nager syndrome is unknown. It is also unclear why affected individuals have bone abnormalities in their arms and legs. Nager syndrome is thought to have an autosomal recessive inheritance pattern when unaffected parents have more than one affected child. The purpose of this report is to present a case of Nager syndrome where the patient exhibited upper limb shortening, an unusual feature that has been reported as coexisting in some individuals with Nager syndrome. CASE REPORT: A 3.5-year-old girl was referred to our Department of Pediatric Otorhinolaryngology due to a cleft palate. Her craniofacial anomalies included malar hypoplasia, severe mandibular hypoplasia with retrognathia, downward slanted palpebral fissures, a high narrow hard palate, absent soft palate, small retroplaced tongue, bilateral external auditory canal atresia, and dysplastic ears. There was no evidence of mental retardation. Based on the craniofacial characteristics and the coexisting upper limb preaxial anomalies, a diagnosis of Nager syndrome was confirmed. CONCLUSION: Nager syndrome is a rare disorder resulting from developmental abnormalities of the first and second branchial arches. It is linked to five other similar syndromes: Miller syndrome, Treacher-Collins, Pierre-Robin, Genee-Wiedemann, and Franceschetti-Zwahlen-Klein. Multidisciplinary management by a craniofacial team is needed. Early intervention, intensive education, new surgical techniques, and an emphasis on coordinated care have improved the quality of life in this patient with Nager syndrome.