[Effects of growth hormone replacement on clinical, radiologic and laboratory parameters improvement in children with vitamin -D dependent rickets [type-2]]

Rickets, which occurs during bone growth development, is a result of severe vitamin D deficiency, that has autosolamal subtypes, i.e. A- type 1- [VDDR-I], due to a defect of the 1-alfa-hydroxilase enzyme and B] type -2[VDDR-II], due to a mutation in the intracellular vitamin -D receptor [VDR], associated with some specific symptoms like alopecia [global head hair loss] and short stature. Diagnosis was established based on physical examination, laboratory findings and radiological examination. We report here 3 cases with vitamin D-dependent rickets, type-II, all members of the same family [2 sisters, aged 1.5, 5 years and their 5 year-old cousin] who had referred to the endocrine and metabolic center in the Imam-Reza hospital in Mashhad, presenting with limb deformities, wide wrist, seizures, disorder in dental growth, alopecia, and several bone fractures. Despite various treatments, they had not recovered. However it was notable that after receiving growth hormone therapy for growth failure, rickets symptoms improved dramatically in clinical, laboratory, and radiological parameters. Using growth hormone therapy to improve height, we observed improvements in the underlying disease of siblings with VDDR-type-II. Growth hormone therapy may be advisable to treat VDDR-type II symptoms

[ effect of culture medium on metabolic and antibacterial activities of probiotic bacteria]

Probiotic bacteria is added directly to food components and it has beneficial effect on function and the health of organisms. The bifidogenic factors enter the colon where they contribute to an increase lactic acid bacteria population including Lactobacilli and Bifidobacteria and they inhibit enteric pathogenic bacterial growth. The aim of this study is to investigate the effect of culture medium on metabolic and antibacterial of probiotic bacteria. In this study, the probiotics bacterial and intestine pathogenic are to be used. Lactobacilli and Bifidobacterium were identified by plating samples on MRS medium, Gram Staining and standard biochemical methods. The effect of antagonistic probiotics was investigated in the presence of growth factor in the method well diffusion Ager on the Shigella flexneri [PTCC 1234], Escherichia coli [PTCC 1552], Salmonella typhi [PTCC 1609] and the culture medium pH was measured. The probiotics bacterial growth in MRS and lactose1%, sorbitol, raffinose, riboflavin were shown the effect antibacterial. The results of the study show the most antagonistic activity in commercial strain Lactobacillus acidophilus on Shigella flexneri and lower activity was in Lactobacillus casei [PTCC 1608], and Salmonella typhimurium [PTCC 1609], and also in Bbifidobacterium bifidum, it showed the most decrease pH value. According to the result of the study, adding growth factors to MRS medium base and lactose 1%, probiotic growth was increased and which also increased antagonistic activity

[Evaluating antimutagenic activity of probiotic bacteria isolated from probiotic products]

Probiotic bacteria are microbial nutrition supplements which have useful effects on human health by maintaining of bowel microbial balance. There are many studies that have been suggested the use of probiotic products as cancer risk reducer. The aim of this study, is isolation and detection of probiotic agents from yoghurt and probiotical tablet and evaluation of their abilities to decrease some effects of mutagenic and carcinogenic agents. In this study, probiotic bacteria were isolated from yogurt and probiotic tablet by using MRS in anaerobic condition [5% Co2 and gas peck] and temperature of 37[degree]c. Then, they were detected by using biochemical tests. Their anti mutagenic effects of supernatant culture were evaluated against mutagenic agents of azid Sodium and Potassium Permanganate by ames test [Salmonella typhimurium TA100] in presence and absence of S[9]. Six probiotic bacteria were isolated from yogurt and probiotic tablet. Their anti mutagenic activity results based on ames test showed they can inhibit mutagenic agents more than 40% in some species, which is considered as a good result. The results of this study show that the use of probiotic bacteria found in different products such as yogurt and probiotic tablets, have proper anti mutagenic and anti carcinogenic effects. They change the micro flora of bowel and, as a result, reduce absorption of mutagenic and carcinogenic agents and help to maintain human health

[Optimizing conditions of lead removal by Saccharomyces cerevisiae in laboratory condition]

Lead enters the human body through water, food, air and accumulates in bones instead of calcium. It can damage nervous system, kidneys and genital system especially in children and also affects hemoglobin synthesis. To optimize the environment conditions to increase the biosorption with Saccharomyces cerevisiae as well as cell immobilization and determination of its efficiency associated with degree of biosorption. This was a cross-sectional study carried out at the Laboratory Center, Islamic Azad University, Sciences and Researches Division, Tehran [Iran] during 2009-2010. The strain of Saccharomyces cerevisiae used in this study was obtained from Lorestan yeast manufacturer, Lorestan, Iran. All factors affecting biosorption including the initial lead concentration, contact time, and pH were investigated. Entrapment method was used to immobilize cells. The maximum biosorption capacity of 80 mg g[-1] dw was observed at the following conditions: contact time 2 h, initial lead concentration 500 ppm, and pH 4.5. The metal biosorption capacity of pretreated yeast strain by autoclave was 35.3 mg g[-1] dw. Also, the biosorption capacity of immobilized cells was almost doubled compared to that of free cells. Regarding the data found in our study, non-pretreated and stabilized Saccharomyces cerevisiae is a suitable biosorbent for removal of lead ions from environment

[Evaluation of bacteria isolated from industrial wastewaters in removal of lead]

Lead is a metal which has the most destructive effect on infants and fetuses. Moreover, it has negative biochemical effects on all persons including the destructive effects on the kidneys, digestive system, intestine, joints, and reproductive system and may cause acute or chronic damage to the nervous system. The aim of this research was screening the bacterial biosorbents with high capacity for absorbing lead among other microorganisms. At first, by using medium containing of determined concentration of Pb [NO[3]][2], resistant bacterial isolates were received from three wastewater samples collected from the cities of Qom and Qazvin. Then, the Minimum Inhibitory Concentration of mentioned isolates was determined by agar dilution method. In order to identify isolates with metal uptake ability, a particular method [Pumpel et al, 1995] was used. At a later stage, biosorption capacity of lead for each isolate with metal uptake ability was investigated by using an Atomic Absorption Spectrophotometer. Finally, after selection and identification of two effective isolates in removal of lead, optimizing the biosorption conditions was carried out in several steps. Out of 29 bacterial isolates that received, 15 lead resistant isolates with MIC >/= 7mM were selected in order to study lead uptake ability that resulted in selection of isolates Q-III and P-II with biosorption values of 162.8 mg g-1dw and 125.6 mg g-1dw, respectively. On the other hand, the maximum biosorption capacity of lead was observed by isolate Q-III in optimum conditions including contact time 2 h, pH 5, wet biomass concentration 0.5g and for isolate P-II in contact time 2 h, pH 5.5, wet biomass concentration 0.4 g. The results of this study indicated that bacterial isolates Bacillus sp. Q-III and Pseudomonas sp. P-II are two suitable biosorbents for the removal of Pb [II]

effect of problem-solving education on self- management and hba1c in adolescents with type 1 diabetes

Background and Purpose: Diabetes mellitus is the most common endocrine disease in children. Achieving selfmanagement of the child and her family is one of the important goals nursing these patients. The present research was conducted to examine the effect of problem solving education on the self-management and HbA1C in adolescents with type 1 diabetes

Methods and Materials: In this experimental study, adolescents 10-19 years old with type 1 diabetes for at least on year, were allocated randomly into two control and problem-solving education groups. The intervention group received 6 sessions of problem-solving process education; the family function of caregivers, self-management and HbA1C of adolescents with type 1 Diabetes before and 3 months after the intervention were evaluated. Data analysis was done using independent and paired t-test, Mann-Whitney U and Wilcoxon tests

Results: Problem-solving education increased self-management in adolescents by 76.57% in terms of public health, 49.11% regimen therapy, 112.20% social presence and 75% in terms of overall self-management [p<0.001]. Problem solving education also decreased HbA1C by 0.62 on average. There was a significant negative correlation between frequency of insulin injections per day and HbA1c levels [p<0.0001]

Conclusion: Problem-solving education is remarkably effective in promoting the self-management in adolescents with type 1 diabetes

[Sinus histiocytosis with massive lymphadenopathy [Rosai-Dorfman disease]: a case report]

Sinus histiocytosis with massive lymphadenopathy [SHML] or Rosai-Dorfman disease [RDD] is a rare benign histiocytic disease of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. In this article case of RDD in a 5-year-old male with large right inguinal and bilateral cervical lymphadenopathies were, occasional fever and anemia was described. The clinical presentation, histopathologic characteristics, and treatments of RDD were discussed

Evalution of lipids level and its relation to HbA1C in diabetic children

Type 1 diabetes is the most common endocrine disorder of childhood. This disease is followed by a variety of biopsycho- social consequences of which cardiovascular diseases are considered to be the most significant cause of mortality among them. This is due to atherosclerosis, and dyslipidemia plays an important role as a risk factor. The aim of the present study was to evaluate the need for early screening of patients for dyslipidemia. In a descriptive and cross-sectional study we assessed a sample of 51 randomly selected patients, with a mean age of 13.47 +/- [2.6] years, ranged from 5 to 19 years, referred to Khorasan Center of Diabetes Research. We determined lipoproteins with biochemical methods and Hemoglobin A1C [HbA1C] with chromatography as well. Finally, the data was analyzed by SPSS. The mean levels +/- ISD of triglycerides [TG], total cholesterol [TC], low density lipoproteins [LDL], high density lipoproteins [HDL], and HbA1C were 97 +/- 55.4, 180.8 +/- 44.1, 113.3 +/- 32.6, 48.4 +/- 10 mg /dl and 8.1% +/- 1.8 respectively. In poorly controlled patients [HbA1C>9], the mean level of TG, TC, LDL, HDL was 123.6 +/- 5.80, 199.40+61.78, 129.20 +/- 44.66 and 50.93+9.61, and in the second group [HbA1C

Congenital adrenal hyperplasia

Congenital adrenal hyperplasia [CAH] is an autosomal recessive disorder caused by any of the enzymatic steps requied to synthesize adrenal hormones. Depending on the enzymatic step that is deficient, there may be clinical and laboratory finding. The aim was to evaluate the epidemiology and clinical finding of CAH. The clinical and laboratory characteristics of 68 patients with CAH who were managed in the pediatric Endocrinology unit of Imam Reza Hospital during 7 years were recorded in this study; the clinical and epidemiological characteristics of congenital adrenal hyperplasia were evaluated in 68 patients admitted Imam Reza Hospital in Mashed, also one patient has been followed in endocrine and metabolism pediatric clinic in this time, during 6 years retrospectively and I year prospectively. Parental consanguinity rate among families of patients was higher than the general population in Mashad [73.8% vs 30%]. In 44.6% of patients the history of disease were positive in sibling. 52.2% of patients were males and 47.8% females. 21- Hydroxylase deficiency was present in 60 patients [88.2%], salt - losing form in 40 [66.7%] and simple virilizing form in 20 [33.3%] of them. 11- beta hydroxylase deficiency was present is [10.3%]. Also one case of 3- beta OHSD was reported. The median chronological age at diagnsis in sall- losing form were 75 and 85 days in boys and girls; in simple virilizing form were 46 and 43 months in boys and girls. 14 of girls were considered to be male before diagnosis was established. This study underscores the necessity for prospective neonatal screening, so that the benefits of early diagnosis and treatment can be realized. In addition the incidence of CAH is expected to be high due to a high rate of consanguinity in our population, here genetic counseling before marriage is beneficial in our population

Report of 3 cases autoimmune polyendocrinopathy [APECED]

Autoimmune polyendocrinopathy syndrome with mucocutaneus candidiasis and ectodermal dystrophy is a rare syndrome which appears in the first decade of life. The first manifestation of this syndrome is mucocutaneus candidiasis in the breast feeding period and the last is adrenal failure. In this study we present 3 cases of APECED syndrome who were admitted in the hospital with convulsion due to hypocalcemia. In physical examination the nails were small and dystrophic, and hare bilateral keratitis, skin almost dry and alopia was also seen. We aimed to show the significant role of poly endocrinopathy type I as a benign cause of hypocalcemia and it's easy treatment with early diagnosis

Severe photophobia in a teenager with APECED syndrome

Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy [APECED] syndrome is a rare, but well-known cause of chronic keratoconjunctivitis with early onset. Patient: A 15 year-old boy with APECED syndrome, who was referred to our hospital because of intense photophobia and blepharospasm. On clinical examination the patient had severe keratitis accompanied by iridocyclitis. The patient showed dramatic response to topical corticosteroids and cycloplegics. Chronic bilateral keratitis is an ocular manifestation of APECED. When an ophthalmologist is confronted with a young child or adolescent and bilateral chronic keratitis with symptoms of intense photophobia, blepharospasm, lacrimation and either with mucocutaneous candidiasis or hypoparathyroidism, APECED is a disease of consideration and appropriate investigation should be done

Report of two cases of idiopathic precocious puberty in family

Precocious Puberty is the result of premature activation of LHRH neuronal function due to structural damage of the neuroendocrine brain systems, or generalized activation of the neuronal glial mechanisms. In this article we present two sisters with idiopathic precocious puberty, one of them recived medication and the other under follow up