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1.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 138-146, 2022.
Article in English | WPRIM | ID: wpr-926872

ABSTRACT

Purpose@#Children with celiac disease (CD) are at an increased risk of low bone mineral density (BMD) owing to malabsorption of fat-soluble vitamins, inflammation, and malnutrition. This study aimed to determine the prevalence and risk factors for low BMD in Iranian children with CD. @*Methods@#This prospective cohort study examined 149 Iranian children with CD between 2011 and 2018 at Zabol University of Medical Sciences. BMD was measured using dualenergy X-ray absorptiometry. Demographic, clinical, and laboratory data were collected from patients’ medical records. Logistic regression analysis was performed to identify the factors associated with low areal BMD (BMD-Z <−2) in the lumbar spine and femoral neck.Descriptive data were analyzed using the mean, standard deviation, and relative frequency.Data were analyzed using the chi-square test, t-test, and analysis of variance. @*Results@#Of the 149 children with CD, 27.5% had osteoporosis. The mean body mass index (BMI) Z score was −1.28±1.2. Lower BMI was associated with a higher likelihood of BMD-Z (odds ratio 2.17; p≤0.05). @*Conclusion@#Overall, the findings of this study showed that there was no correlation among Marsh classification, presence of specific human leukocyte antigens, and low BMD in Iranian children with CD. BMI can be a predictor of bone density in children with CD and may be applied clinically in early screenings to evaluate the bone health status in these children.

2.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 197-206, 2021.
Article in English | WPRIM | ID: wpr-903100

ABSTRACT

Purpose@#Celiac disease (CD) is a common autoimmune disease with extra-intestinal manifestations, including neurological disorders. There are few reports to assess various factors in increasing the chances of developing neurological disorders in CD, so we designed this study. @*Methods@#All patients with CD at any age who had been referred to the Celiac Clinic were evaluated for neurological problems. CD was defined as IgA anti-transglutaminase antibodies (anti-tTG) of 18 IU/mL or higher in serology and Marsh type I or more severe in histopathological evaluation. Logistic regression analysis was used to evaluate the impact of various independent variables on the neurological manifestations. @*Results@#A total of 540 patients enrolled in this study. A 360 (66.7%) of patients were children. A 64.8% and 35.2% were female and male, respectively. Overall, 34.1% of patients had neurological manifestation, including headache, neuropathy, epilepsy, and ataxia. The odds of developing neurological manifestations in children were significantly lower than in adults (odds ratio [OR], 0.66; 95% confidence interval [CI], 0.45–0.96; p=0.03) and in patients with gastrointestinal (GI) symptoms significantly higher than in the group without GI manifestations (OR, 1.77; 95% CI, 1.18–2.63; p=0.005). Other variables, including Marsh classification (OR, 0.44; 95% CI, 0.18– 1.11; p=0.08) and anti-tTG levels (OR, 1.00; 95% CI, 0.999–1.001; p=0.59) did not significantly increase the chances of developing neurological disorders. @*Conclusion@#Our study showed that increasing age and the presence of GI symptoms, but not serological and histological findings, could increase the chances of developing neurological diseases in CD patients.

3.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 197-206, 2021.
Article in English | WPRIM | ID: wpr-895396

ABSTRACT

Purpose@#Celiac disease (CD) is a common autoimmune disease with extra-intestinal manifestations, including neurological disorders. There are few reports to assess various factors in increasing the chances of developing neurological disorders in CD, so we designed this study. @*Methods@#All patients with CD at any age who had been referred to the Celiac Clinic were evaluated for neurological problems. CD was defined as IgA anti-transglutaminase antibodies (anti-tTG) of 18 IU/mL or higher in serology and Marsh type I or more severe in histopathological evaluation. Logistic regression analysis was used to evaluate the impact of various independent variables on the neurological manifestations. @*Results@#A total of 540 patients enrolled in this study. A 360 (66.7%) of patients were children. A 64.8% and 35.2% were female and male, respectively. Overall, 34.1% of patients had neurological manifestation, including headache, neuropathy, epilepsy, and ataxia. The odds of developing neurological manifestations in children were significantly lower than in adults (odds ratio [OR], 0.66; 95% confidence interval [CI], 0.45–0.96; p=0.03) and in patients with gastrointestinal (GI) symptoms significantly higher than in the group without GI manifestations (OR, 1.77; 95% CI, 1.18–2.63; p=0.005). Other variables, including Marsh classification (OR, 0.44; 95% CI, 0.18– 1.11; p=0.08) and anti-tTG levels (OR, 1.00; 95% CI, 0.999–1.001; p=0.59) did not significantly increase the chances of developing neurological disorders. @*Conclusion@#Our study showed that increasing age and the presence of GI symptoms, but not serological and histological findings, could increase the chances of developing neurological diseases in CD patients.

4.
Gastroenterology and Hepatology from Bed to Bench. 2017; 10 (1): 70-72
in English | IMEMR | ID: emr-185424

ABSTRACT

Lithophagia is a type of pica that might be resulted from Iron Deficiency Anemia [IDA]which is the frequent presenting signs of Celiac Disease [CD]. A 5-year-old child with a two year history of the lithophagia with a, refractory IDA, abdominal distention and constipation. The child did not grow well and had failure to thrive. With suspicion to CD, TTg IgA level was measured and due to an incearse of TTg IgA level the patients were undergone esophagogastrodeudonoscpy and jejunal biopsy. The biopsy showed severe villous atrophy and an increase in limphoplasma cells. Biopsy confirmed diagnosis of CD and glutten free diet was initiated finally. Six months after diagnosis and commencing the gluten free diet, the lithophagia and constipation in patient eradicated completely. IDA and failure to thrive were improved and the level of TTg IgA was reached to the normal. The case demonstrated the relationship between lithophagia and CD in anemia. Therefore, in the same cases such as our case should be considered CD as the most important causes of lithophagia

5.
Gastroenterology and Hepatology from Bed to Bench. 2016; 9 (1): 64-67
in English | IMEMR | ID: emr-174985

ABSTRACT

A common belief is that alkali ingestion causes severe esophageal damage and limited gastric injury due to the buffering action of acid. Gastric injury has been observed in patients who ingested alkali. Gastric outlet obstruction [GOO] secondary to caustic ingestion occurs due to fibrosis after resolution of the acute injury and inflammation, most commonly 6 to 12 weeks after initial ingestion. The traditional treatment for GOO related to ingestion of corrosive agents is surgery. Experience with endoscopic balloon dilation of corrosive-induced GOO is limited in children. This is the first report of endoscopic pyloroplasty in a child with GOO due to caustic alkalis ingestion that was treated with balloon dilation [using TTS balloon ranging from 6-15 mm] in Iran. Four dilation sessions were required for symptomatic relief of dysphagia. After one year of follow up, weight gain was normal


Subject(s)
Infant , Humans , Endoscopy, Gastrointestinal , Alkalies , Eating , Child
6.
Govaresh. 2016; 21 (3): 188-192
in English | IMEMR | ID: emr-185876

ABSTRACT

Background: Drug abusers are one of the most at risk populations for hepatitis C virus [HCV] transmission worldwide. The aims of this study were to measure the seroprevalence of HCV and to compare certain related risk factors in participants who were referred to drug rehabilitation centers affiliated to Shiraz University of Medical Sciences, Shiraz, Iran


Materials and Methods: Blood samples and interviews containing questions about age, sex, level of education, house status, jobs, history of imprisonment and psychiatric problems, age at the first drug and first intravenous [IV] drug uses, safe and unsafe sexual activity, and time and duration of IV drug use in the past 30 days were obtained from 1116 participants in rehabilitation centers. The sera were tested for anti-HCV antibody using enzyme immunoassay. The data were analyzed using independent samples t test and one way ANOVA for quantitative variables and Chi-square and Fisher's exact tests for qualitative variables


Results: Among the 844 participants who agreed to blood sampling, the prevalence of HCV infection was 14.2%. The significant positive associations were detected between anti-HCV antibody positivity and higher levels of education [p =0.008], no history of imprisonment [p<0.001], having a job [p =0.006], having a partner [p <0.001], and higher age at the first drug use [p<0.001]


Conclusion: The seroprevalence of HCV infection among drug abuser was very high in comparison with the general population of Iran. Making policies to prevent transmission of HCV infection among this high risk subpopulation is highly recommended

7.
Gastroenterology and Hepatology from Bed to Bench. 2015; 8 (2): 153-159
in English | IMEMR | ID: emr-178190

ABSTRACT

This study reports evaluated prevalence of CD in patients with Beta-thalassemia major. Celiac Disease [CD] is an autoimmune disorder triggered by ingestion of gluten in genetically predisposed individuals. In this case-control study in a period of 3 years, which was performed on 620 children in two groups of Beta-thalassemia major patients [n=200] and control [n=420], serum tissue transglutamianse [tTG] IgA levels were measured. The two groups were compared together in terms of tTG IgA levels, and p<0.05 was considered significant. The means of serum tTG IgA levels in patients with Beta-thalassemia major and control groups were 28.81+/-68.44 and 6.94+/-6.68 U/mL, respectively. There was a significant difference in favor of the case group [p=0.000]. Body mass index in the two case and control groups had a significant difference [t=3.859, p=0.001]. Belonging to each group will change the probability of having less than 20 in tTG IgA [odds=0.285] and it means that belonging to the control group has a protective role. There is only a significant association in the case of all population [r=0.102, p=0.011]. Body mass index in the two case and control groups had a significant difference [t=3.859, p=0.001]. Probability of CD should be considered since the prevalence of CD is high in patients with and Beta-thalassemia major. Patients with thalassemia major are recommended for screening for CD


Subject(s)
Humans , Male , Female , beta-Thalassemia , Prevalence , Serology , Case-Control Studies , Transglutaminases , GTP-Binding Proteins , Immunoglobulin A , Body Mass Index
8.
Middle East Journal of Digestive Diseases. 2015; 7 (1): 31-35
in English | IMEMR | ID: emr-155017

ABSTRACT

Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and pen-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 [Chicago, IL, USA]. Of 222 children with functional constipation, 124[55.9%] were girls and 98 [44.1%] were boys with a mean +/- SD age of 5 +/- 3.12 years. The mean +/- SD duration of constipation was 2.2 +/- 1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls [p<0.01]. Fecal soiling was present in 40.8% of the boys and 28.2% of the girls [p=0.04]. Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group

9.
Gastroenterology and Hepatology from Bed to Bench. 2015; 8 (1): 42-48
in English | IMEMR | ID: emr-152943

ABSTRACT

The aim of this study is to find-out the possible etiologies in Iranian infants less than three months in Shiraz, South of Iran. Cholestatic jaundice most probably occurs due to a pathological condition and the most frequent causes in early infancy are neonatal hepatitis and biliary atresia. Early diagnosis and treatment of infantile cholestasis can improve prognosis of liver diseases by prevention of the complications of these disorders. In this retrospective study, 122 infants under 3 months of age with cholestasis were studied in Nemazee Hospital [affiliated to Shiraz University of Medical Sciences] during the years 2001-2011. Demographic data, duration of jaundice, liver biopsy and the causes of cholestasis were recorded. There were 76 males [62.3%] and 46 females [37.7%] with a mean age of 54.4 +/- 23.7 days. The most common clinical finding was jaundice that was seen in all patients [100%].The onset of jaundice was the first day to the fifty two days of age, with an average age of 15.6 +/- 16.1 days. Other findings included hepatomegaly in 92 patients [76.4%], clay-color stool in 54 [44.3%], and splenomegaly in 29 patients [23.8%]. In this study, the most common causes of cholestasis were biliary atresia [30=24.6%], idiopathic neonatal hepatitis [30= 24.6%] and bile ducts paucity [16=10.3]. The results of this study showed that biliary atresia and neonatal hepatitis are the most common cause infantile cholestasis in this area. It is recommended that biliary atresia should be discriminated from other form of neonatal cholestasis

10.
Gastroenterology and Hepatology from Bed to Bench. 2015; 8 (4): 270-277
in English | IMEMR | ID: emr-173161

ABSTRACT

This study aimed to evaluate the erythromycin efficacy in childhood cyclic vomiting syndrome. Cyclic vomiting syndrome [CVS] is an unusual cause of episodic emesis in children and erythromycin is an effective treatment. In this prospective study, 301 patients with a final diagnosis of CVS enrolled in two separated groups. The first group received erythromycin for 7 days and propranolol for at least 9 months [n=155]. The second group was treated with propranolol alone for at least 9 months [n=146]. These two groups were compared for response to the treatment and the recurrence of symptoms after treatment completion. Relationship of response, recurrence, and characteristics of the disease was assessed. Both groups showed a significant difference in terms of response to treatment [P=0.002], however the recurrence after treatment completion had no considerable difference [P=0.563]. There was no relationship between CVS characteristics and these two items [response and recurrence]. In our point of view, the addition of erythromycin to standard propranolol treatment can improve the response to treatment, although it has no significant effect on recurrence of CVS symptoms. We suggest the use of erythromycin for 7 days in addition to CVS standard therapy

11.
Middle East Journal of Digestive Diseases. 2015; 7 (2): 94-99
in English | IMEMR | ID: emr-166787

ABSTRACT

Due to the increased prevalence of celiac disease in chromosomal anomalies and other congenital anomalies, this study was conducted to evaluate the seroprevalence of celiac disease [CD] in patients with congenital heart defects [CHD]. This case-control study was done on 1002 children in two groups of CHD patients [n=402] and controls [n=600]. The serum tissue transglutamianse [TTG] levels were investigated. The two groups were compared in terms of TTG IgA levels and p<0.05 was considered as the significant level. The means of serum TTG IgA levels in children with CHD and the control groups were 19.17 +/- 46.67 and 7.77 +/- 10.02 u/mL respectively [p=0.001]. After ANOVA analysis a significant difference between two cyanotic and acyanotic subgroups of cases and control groups was observed [p=0.000]. The follow up tukey test showed only non-significant difference between the cyanotic and acyanotic cases. The frequency of TTG IgA with the consideration of 20 u/mL as cut-off point showed a significant association with groups [X2=28.31 and p=0.000]. According to the results the serum TTG IgA levels were significantly higher in patients with CHD than normal children and screening for CD in children with CHD is recommended


Subject(s)
Humans , Heart Defects, Congenital , Case-Control Studies , Serologic Tests , Child , Prevalence
12.
Arab Journal of Gastroenterology. 2015; 16 (3-4): 90-93
in English | IMEMR | ID: emr-174959

ABSTRACT

Background and study aims: Liver biopsy is a well-established procedure in the diagnosis and follow-up of liver diseases. Complications of liver biopsy are rare but potentially lethal. The aim of this study was to evaluate the complications of percutaneous liver biopsy and to compare the complications of blind and ultrasound-guided percutaneous liver biopsy in paediatric wards of Nemazee Hospital of Shiraz in the south of Iran


Patient and method: To complete the questionnaire, registered information of liver biopsies due to different causes in paediatric patients between 2008 and 2012 was retrospectively reviewed. All children aged between 0 and 18 years, who underwent liver biopsy [due to any indication], participated in this study


Results: Liver biopsies were obtained from 210 patients. Seven of 210 cases were excluded due to unreliable data. A total of 209 liver biopsies were done in the rest of the cases [n = 203]. Of all cases of liver biopsies, 22 [10.5%] experienced complications after biopsy. Pain [n = 7] was the most frequent complication in 22 cases of liver biopsy. Mortality rate was one [0.5%] due to rupture of subcapsular haematoma. In terms of complication [p = 0.592], there was no significant difference statistically between patients with blind liver biopsy [n = 16] and patients with ultrasound-guided liver biopsy [n = 6]


Conclusion: In terms of complications, there was no significant difference when the patients were evaluated with and without ultrasound-guided biopsy


Subject(s)
Humans , Infant , Infant, Newborn , Child , Child, Preschool , Adolescent , Liver/pathology , Biopsy , Liver Diseases/diagnostic imaging , Liver Diseases/complications , Surveys and Questionnaires , Child
13.
Middle East Journal of Digestive Diseases. 2015; 7 (3): 166-169
in English | IMEMR | ID: emr-166605

ABSTRACT

Celiac disease is an autoimmune mediated small intestine inflammation which occurs due to hypersensitivity reaction to gluten and related proteins in diet in genetically predisposed individuals. Prevalence of celiac among the population is about 0.5 - 1 % in most countries. Frequency of celiac disease in children is the subject of a few research. In this study, we aim to determine the frequency of celiac disease in patients presenting with functional constipation. This cross-sectional study was conducted on children referring to Imam Reza Clinic, affiliated to Shiraz University of Medical Sciences during one year starting from 2011, March 20. One hundred and one children 2-18 years of age with constipation for more than 2 months according to ROME III criteria. The entire participants underwent serologic studies of Total IgA and IgA TTG. Serum IgG TTG was measured in cases with reported values of Total IgA below the lowest normal limits. Moreover, endoscopic biopsy of the small intestine was also performed for patients with positive serology. Of all the 101 studied participants, only four individuals [3.96 %] had positive test results for IgA TTG [potential celiac disease]. One of these patients refused to do endoscopy and endoscopic small intestine biopsy was performed for 3 patients. Two of them had normal pathology and one of them [0.99 %] was confirmed for celiac disease. The frequency of celiac disease in children with chronic constipation is slightly higher than general population but without significant difference 0.99% VS 0.6% ; p=0.64]. So the screening serologic test for celiac disease is not recommended in children with chronic constipation


Subject(s)
Humans , Adolescent , Child , Child, Preschool , Constipation , Cross-Sectional Studies , Child , Chronic Disease
14.
Gastroenterology and Hepatology from Bed to Bench. 2013; 6 (4): 183-189
in English | IMEMR | ID: emr-140125

ABSTRACT

The aim of this study was to describe our results and investigate the survival of below-18-year-old patients undergoing LRDLT and the factors affecting this. Living Related Donor Liver Transplantation [LRDLT] has become a good option to provide suitable grafts for children with liver diseases. Using this method, children who have no chance for life can live a much longer life. The present study is a historical cohort study carried on 191 patients below-18-years-old who had undergone LRDLT for the first time in the Namazi hospital liver transplantation center. Survival rate of the patients was assessed using Kaplan-Meier method. The effect of factors related to the recipients, donors, and the transplantation process on the patients' survival was also investigated. 1, 3, 5 and 11-year survival of patients was 71%, 66%, 65%, and 65%, respectively. In the univariate analysis, age, weight at transplantation, PELD/MELD score, existence of post-transplant complications were found to be effective factors on the patients' survival. In the multivariate analysis, weight at transplantation, PELD/MELD score, and existence of post-transplant complications were the prognostic variables. LRDLT is now well established with satisfactory results in our center. Although the survival rate of the patients is lower than the survival rate reported in other studies, but the survival of the patients who had survived 1 month after the transplantation was comparable to other studies

15.
Iranian Journal of Pediatrics. 2013; 23 (3): 302-308
in English | IMEMR | ID: emr-143196

ABSTRACT

Autoimmune hepatitis [AIH] is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH. The medical records of 87 children [56 girls and 31 boy] diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome. The mean age of the patients was 10.1 +/- 4.5 years [64.4% females]. The most common clinical findings were jaundice [70.1%], splenomegaly [67.8%], and hepatomegaly [51.7%]. Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively [36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases]. The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 [74.7%] patients. The complete response was seen in 52 [59.8%] patients and 24 [27.6%] patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients. AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice


Subject(s)
Humans , Male , Female , Child , Tertiary Care Centers , Retrospective Studies , Autoantibodies , Liver Transplantation
16.
Middle East Journal of Digestive Diseases. 2013; 5 (1): 41-46
in English | IMEMR | ID: emr-130194

ABSTRACT

Liver cirrhosis is one of the major causes of hospitalization and mortality in children. A wide spectrum of disorders including developmental abnormalities, infections, metabolic and genetic disorders can lead to liver cirrhosis in pediatric patients. Determination of its etiology is important for treatment modality, prevention of progressive liver damage, family counseling and prioritizing liver transplantation. The aim of this study is to evaluate the causes of liver cirrhosis in children in Southern Iran. We included all cirrhotic children aged less than 18 years who referred to an outpatient Pediatric Gastroenterology Clinic affiliated with Shiraz University of Medical Sciences between March 2009 and September 2010 in this cross-sectional study. The etiology of cirrhosis was determined according to clinical findings, laboratory tests, radiographic evaluation such as ultrasonography or computed tomography scan, hepatobiliary scintigraphy and histopathologic examination of the liver biopsy. Cirrhosis with unknown etiology was considered as cryptogenic. A total of 106 cirrhotic children aged between 5 months to 18 years old with a mean age of 8.24 +/- 6.12 years that included 60 boys [56.6%] and 46 girls [43.4%] were enrolled in the study. The most common causes of liver cirrhosis were Wilson disease [n=22; 20.7%], biliary atresia [n=19; 17.9%], and cryptogenic cirrhosis [n=14; 13.2%]. Other causes were autoimmune hepatitis [n=12; 11.3%], idiopathic neonatal hepatitis [n=10; 9.4%], hepatorenal tyrosinemia [n=9; 8.5%], glycogen storage disease [n=6; 5.7%], and progressive familial intrahepatic cholestasis [n=4; 3.8%].Considering the most common etiology of liver cirrhosis in children in this part of Iran we suggest testing for Wilson disease in all cirrhotic children


Subject(s)
Humans , Female , Male , Liver Cirrhosis/complications , Liver Transplantation , Hepatolenticular Degeneration , Biliary Atresia
17.
Archives of Iranian Medicine. 2013; 16 (1): 23-24
in English | IMEMR | ID: emr-130529

ABSTRACT

alpha-1 antitrypsin [AAT] deficiency is one of the most important genetic causes of childhood liver diseases in some parts of the world, but its geographic distribution is highly variable. There are many reports from Asian countries such as India, the Philippines, and China which show a very low incidence of this disease. However few studies exist from Iran regarding this genetic deficiency as the cause for prolonged neonatal jaundice. In this study we attempt to investigate the possible role of AAT deficiency as a cause of prolonged neonatal jaundice in the largest pediatric referral center of Southern Iran. We included 126 neonates with the clinical diagnosis of neonatal cholestasis in this study. Polymerase chain reaction-restriction fragment length polymorphism [PCR-RFLP] was performed on the extracted DNA from their blood samples. DNA sequencing confirmed the results of the PCR-RFLP tests. All patients were genetically normal regarding level of AAT, i.e., all were MM homozygotes. AAT deficiency is a rare disease in Iran and is not a major cause of neonatal cholestasis in this country


Subject(s)
Humans , Female , Male , Incidence , Cholestasis , Infant, Newborn , Jaundice, Neonatal
18.
IJPM-International Journal of Preventive Medicine. 2013; 4 (12): 1380-1385
in English | IMEMR | ID: emr-138120

ABSTRACT

Hereditary tyrosinemia type 1 [HT1] is a rare autosomal recessive inborn error of metabolism caused by deficiency of fumarylacetoacetate hydrolase enzyme. This disease manifests with severe liver and kidney impairment and is associated with an increased risk of liver cancer. The aim of this study was to evaluate clinical, laboratory, imaging, and histopathologic characteristics in the children with HT1 who had referred for liver transplantation. The present retrospective study was conducted on 45 children with HT1 who had referred to Organ Transplantation Center affiliated to Shiraz University of Medical Sciences between March 2005 and March 2010. There were 64.4% boys and 35.6% girls with mean age of 3.75 +/- 1.28 year [ranges from 2 months to 13 years]. The most first clinical presentation was hepatic [80%] and the most prevalent physical findings were hepatomegaly [57.8%], splenomegaly [51.1%], ascites [42.2%], and jaundice [37.9%]. The most relevant laboratory parameters were the high serum succinylacetone, alpha-fetoprotein, and tyrosine levels. The most common findings in the patient's abdominal ultrasonography were multiple hepatic nodules [75.6%] and inhomogeneous parenchymal echogenicity of liver [48.9%], while hyper and hypo attenuated nodules [60%] and non-homogeneous pattern of liver parenchyma [53.3%] were the most prevalent findings in abdominal computed tomography scan. In the histopathology of the liver, the most important finding was cirrhosis in all the patients. In this study, 14 patients [31.1%] received Nitisinone [2-[2-nitro- 4-trifluoromethylbenzoyl]-1,3-cyklohexanedione; NTBC]. This study described clinical and laboratory findings in the children with HT1 who had referred for liver transplantation because of end-stage liver disease from all over country, which indicates delay in diagnosis and treatment of this disease. Considering the results of this study, newborn screening for this disease is highly suggested


Subject(s)
Humans , Female , Male , Liver Transplantation , Retrospective Studies , Child
19.
Middle East Journal of Digestive Diseases. 2012; 4 (2): 102-106
in English | IMEMR | ID: emr-178466

ABSTRACT

There are great variations in the incidence and prevalence of inflammatory bowel diseases [IBD] among different populations. Epidemiologic studies mainly come from North America and Europe. Studies from Iran are mostly data on the adult population from the northern region of the country. Medical records of 37 pediatric patients [? 18 years of age] admitted in the Pediatric Gastroenterology Ward at Nemazee Hospital, from 2001 through 2007 with final diagnoses of IBD were reviewed regarding data such as clinical manifestations and colonoscopic findings. There were 19 boys [52%] and 18 girls [48%] with a mean age of 10.3 +/- 4.9 years [range: 2-17 years]. Of these, 26 [70%] had ulcerative colitis [UC], 9 [25%] were diagnosed with Crohn's disease [CD], and 2 [5%] were labeled as indeterminate colitis [IC]. Bloody stools [84.6%] and pallor [80.8%] were the most common features in UC whereas growth failure [88.9%] followed by pallor [77.8%] were the most frequent symptoms in patients with CD. The most frequent colonoscopic findings in UC and CD were erythema [80%] and ulcer [71.4%] respectively. This study provides available epidemiologic data on pediatric patients with IBD from Southern Iran


Subject(s)
Humans , Female , Male , Pediatrics , Crohn Disease/epidemiology , Colitis, Ulcerative/epidemiology , Child
20.
Middle East Journal of Digestive Diseases. 2012; 4 (4): 206-210
in English | IMEMR | ID: emr-149472

ABSTRACT

All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. In previous studies, extra-hepatic disorders have been reported as the main cause of portal hypertension in children. In this study, we investigate the underlying causes of portal hypertension in children. This prospective, descriptive study investigated the etiology of 45 children with portal hypertension who referred to Nemazee Hospital Pediatric Gastroenterology Ward from 2005 to 2007. The underlying causes of portal hypertension were determined by liver biopsy, abdominal sonography, abdominal computed tomography scan, and liver Doppler sonography. In this study, 42 of 45 patients [93.3%] developed portal hypertension due to intrahepatic diseases. Extra-hepatic portal hypertension was detected in 3 [6.7%] patients with portal vein thrombosis. Intrahepatic diseases were the most common etiology of portal hypertension among children who referred to our center.

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