Response surface methodology-based optimization of ultrasound-assisted extraction of β-sitosterol and lupeol from astragalus atropilosus (roots) and validation by HPTLC method

Objective: To optimize the ultrasonication method for efficient extraction of P-sitosterol and lupeol from the roots of Astragalus atropilosus using Box-Behnken design of response surface methodology (RSM), and its validation by high performance thin layer chromatography (HPTLC) method. Methods: Ultrasonication method was used to extract β-sitosterol and lupeol from Astragalus atropilosus (roots). RSM was used to optimize the different extraction parameters viz. liquid to solid ratio (10-14 mL/g), temperature (60-80 °C) and time (40-60 min) to maximize the yield of β-sitosterol and lupeol. The quantitative estimation of β-sitosterol and lupeol was done in chloroform extract of Astragalus atropilosus by validated HPTLC method on 10 cm × 20 cm glass-backed silica gel 60F

Screening for autoimmune diseases in type 1 diabetes: low incidence of adrenal insufficiency

Primary Adrenocortical insufficiency [Addison's disease] is a potentially fatal condition that often develops incidentally and can be easily overlooked. Although rare in the general population, it is more common in patients with type 1 diabetes mellitus [T1D]. In this study, we reviewed our experience with the occurrence of associated adrenal insufficiency [AI] in children with T1D over 15 year's period at King Khalid University Hospital [KKUH], Riyadh, Saudi Arabia. This is a retrospective hospital based study, included children and adolescents with T1D at KKUH in the period January 1995-December 2012. All patients were serologically screened for Celiac and thyroid diseases. Adrenal function was assessed at the time of diagnosis, and annually thereafter by measuring serum cortisol and adrenal corticotrophic hormone [ACTH] using the available commercial kit. Adrenal cortex antibodies [AAA] test was done by Bioscientia laboratory, Germany, in one patient. In a cohort of 305 children and adolescents with T1D at KKUH, only one patient was found to have AI as a part of autoimmune polyendocrine syndrome type 1. Thyroid functions were abnormal in 65 [21.3%] patients. Of these, 26 [8.5%] patients have evidence of overt hypothyroidism and 39 [12.8%] patients had subclinical hypothyroidism. In twenty-six patients [8.5%], the intestinal biopsy results were positive for CD. There is no international consensus on the issue of screening for AI in children with T1D. In our experience, we do not favour screening for AI in children with T1D unless there is a clear risk factor

Celiac disease in children with short stature: a hospital based study

To identify the prevalence of celiac disease [CD] among children with short stature. Celiac disease [CD] is genetically determined gluten-sensitive enteropathy resulting in nutrient malabsorption, with an increasing incidence world-wide. A retrospective, hospital based study is conducted at a pediatric endocrine clinic, King Khalid University Hospital, Riyadh. During the period between January 1990 and December 2009, the medical records of patients evaluated for short stature were reviewed. After a proper detailed history and physical examination, growth analysis, followed by radiological [bone age], and laboratory screening [CBC, and thyroid function] were performed. Celiac serological screening and chromosomal analysis were performed when appropriate, followed by small intestinal biopsy if indicated. Growth hormone stimulation test was performed in suspected patients, followed by magnetic resonance imaging [MRI] in positive cases. Hundred and ten patients were evaluated for short stature over the period under review. Their age ranged from 2.6 to 14 years. The male to female ratio was 1.3:1. The commonest cause was genetic and found in approximately 51.8%, while in the other 48.2%, variable endocrine and non-endocrine causes were noted. Of the non-genetic short stature, 5 [10%] patients were found to have celiac disease, i.e. 4.5% of short children. The prevalence of celiac disease is not rare in this study, therefore, celiac disease must be considered as an important cause of short stature. As, it could be a silent disease, it is recommended that a serological screening be done first to all patients before performing dynamic growth hormone testing in the evaluation of short stature

Spectrum of endocrine disorders at the paediatric endocrine clinic, king khalid university hospital, Riyadh, Kingdom of Saudi Arabia

Paediatric endocrinology is a well-established, expanding sub-speciality; therefore, paediatricians must be trained to meet the increasing requirements. The aim of this study was to present the pattern of endocrinopathy seen in a specialized clinic. A retrospective review was conducted of cases of endocrine disorders seen between December 1989 and December 2009 at the Paediatric Endocrine Clinic, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia. Rickets was common at all ages [99 patients], the main cause being nutritional. Ambiguous genitalia were also common [81 patients]; congenital adrenal hyperplasia was the predominant cause [96%] in 46 XX genetic females. Short stature was a frequent reason for referral [68 patients], with familial short stature as the commonest cause [54.4%]. Obesity was diagnosed in 43 patients and was due to nutritional factors in 72% of cases. Central diabetes insipidus was found as an isolated autosomal dominant condition and also in conjunction with other hormonal deficiencies. Carbohydrate metabolic dysfunction was common in infants and children, with an increasing prevalence of type 1 diabetes mellitus. A programme to detect neonatal congenital hypothyroidism was effective in minimizing sequel; congenital hypothyroidism was diagnosed in 16 patients before the programme, and 134 cases were detected by screening. Graves disease was diagnosed in five patients and Hashimoto thyroiditis in 20 patients, associated with type 1 diabetes mellitus in six and Down syndrome in three. The frequency and pattern of endocrine disorders indicate the need for well-trained paediatricians to improve the health of the population.

Emergency autologous vein graft reconstruction after using a vascular closure device / Reconstrução de emergência de enxerto autólogo de veia após uso de dispositivo de fechamento vascular

An emergency operation for access related acute critical limb ischemia with signs of infection is described. Inguinal femoral reconstruction was performed with a bifurcated graft constructed from the ipsilateral saphenous vein.

Uma operação de emergência relacionada à isquemia aguda com sinais de infecção é descrita. Reconstrução femoral inguinal foi realizada com um enxerto bifurcado feito a partir da veia safena ipsilateral.

Pseudohypoparathyroidism type 1b; a rare cause of femoral neck fracture

A 6-year-old boy was seen in general pediatric clinic for assessment of possible rickets. He had hypocalcaemia and hyperphosphatemia. Serum calcium 1.47 mmol/L, phosphorus 2.5 mmol/L and alkaline phosphatase 1957 IU/L were recorded. Radiological X-ray of the hip showed multiple lytic lesions. The diagnosis of rickets was postulated with secondary hyperparathyroidism and was started on Vitamin D3 and oral calcium. He was treated for rickets at the age of one year. Parents were consanguineous. At the age of seven years, he presented to the emergency room with left femoral neck fracture following a minimal trauma which required open reduction and internal fixation. Physical examination revealed no dysmorphic features. Biochemical investigations revealed normal complete blood count, liver and renal functions and arterial blood gas. However, serum calcium was low 2.0 mmol/L, phosphorous 2.1 mmol/L and alkaline phosphatase 1752 IU/L, serum PTH was high 1406 ng/L with normal 25 [OH] Vit. D3 and 1,25 [OH][2] Vit. D3. Pseudohypoparathyroidism [PHP] is an uncommon metabolic bone disorder characterized by biochemical hypoparathyroidism [i.e.,hypocalcaemia and hyperphosphataemia], increased secretion of parathyroid hormone [PTH], and target tissue unresponsiveness to the biological actions of PTH. In addition, many patients with PHP exhibit a distinctive constellation of developmental and skeletal defects. Pseudohypoparathyroidism type 1b can be presented as skeletal fractures. We highlight the importance of this rare cause in differential diagnosis

Ambiguous genitalia: two decades of experience

Ambiguous genitalia is a complex, medical and social emergency. The aim of this study is to present our experience over two decades, focusing on the pattern and clinical presentation. A retrospective study conducted in the pediatric endocrine clinic at a university hospital in Saudi Arabia during the period 1989-2008. Medical records of children with ambiguous genitalia were reviewed and the genitalia described. Of the 81 children with ambiguous genitalia, 53 [65.4%] patients were genetically females [46XY], with congenital adrenal hyperplasia being the common cause in 51 [96.5%] patients. Hyperpigmentation, variable degrees of salt wasting and a family history of a similar problem helped in diagnosis. Male genetic sex [46XY] was present in only 28 [34.6%] patients with a diversity of causes; multiple congenital anomalies in 9 [32.1%], local anorectal anomalies in 2 [7.1%], congenital adrenal hyperplasia [3-[beta-hydroxysteroid dehydrogenase deficiency] in 2 [7.14%], 5-alpha-reductase deficiency in 4 [14.28%], partial androgen insensitivity in 3 [10.7%], complete androgen insensitivity in 4 [14.28%], and hypogonadotrophin deficiency in 4 [14.3%]. Twenty-five [47.2%] of females were wrongly assigned as males, where only two [7.1%] males were wrongly assigned as females. Ambiguous genitalia, currently termed disorders of sex development [DSD], is not uncommon in our community. Increased awareness, a detailed history, and a careful physical examination, coupled with appropriate laboratory and radiological investigations aid in early diagnosis and avoid serious sequelae

Outcomes of surgical treatment of malrotation in children

Background: Urethral mucosal prolapse is rare. This condition may be confused with tumour or sexual abuse in girls. This study aims at reporting the pathology presentation and therapeutic options of urethral prolapse in girls. Materials and Methods: A retrospective study was undertaken from January 2000 to December 2008. Authors analysed the clinical features and the treatment options. Results: There were nine cases of urethral prolapse. The ages ranged from 2.5 to 10 years (mean age: 5.08 years). The main presentation was vaginal bleeding (five cases). Physical examination revealed a soft; non-tender mass that bleeds on touch (six cases); with a length ranging from 0.75 to 1 cm. Urine culture in four patients revealed urinary infection that yielded Escherichia coli in three cases and the Staphylococcus aureus in one case. Six patients had surgical treatment while three had medical treatment. In those who had surgery; one had acute urine retention and one had recurrence that was treated successfully without operation. All the nine girls are cured. Conclusion: Urethral prolapse is a disease of the prepubertal girls of low socio-economic group. Diagnosis is clinical. The treatment of choice is surgical

Pilgrims satisfaction with ambulatory health services in Makkah, 2008

The main objective of this study was to assess the level and correlates of patients' satisfaction with ambulatory health services provided for pilgrims during Hajj period in 2008. This was a facility-based, cross-sectional study conducted in the Makkah region during the Hajj season in December 2008. A two-stage technique was used to select 500 patients from those who attended the ambulatory health services. One hundred subjects were selected by systematic random sampling [every fifth] from each of the five hospitals included in the study and asked to fill in a pilot-tested self-administered questionnaire. A total of 487 questionnaires were analyzed. Descriptive statistics and t-test, Mann Whitney test and ANOVA, or Kruskal-Wallis test was used as appropriate after checking for normality. Level of significance level was set to be <0.05 throughout the study. From 478 subjects analyzed, 390 [81.6%] were man, 345 [72.2%] were married, 28.9% had either intermediate or high secondary school education, and 2.4% were skilled laborers. The total satisfaction score for health facilities was 20.45 +/- 4.03 of 25. The satisfaction scores were 20.15 +/- 4.7 of 25 for patient satisfaction with physicians and 21.35 +/- 4.5 for patient satisfaction with paramedical personnel. The overall satisfaction score was 61.5 +/- 4.5 of 75 points. There were significant relations between total satisfaction of health facilities with education level and with occupation [P = 0.012, 0.001, respectively]. The total satisfaction of patients with physicians was significant only with education level. The overall satisfaction score had a significant relation with occupation [P = 0.03], but a borderline relation with the education level [P = 0.056]. Satisfaction with ambulatory Hajj health services is acceptable. Some physicians and waiting area services need special attention to improve satisfaction levels with ambulatory health in the subsequent Hajj seasons

Ambiguous genitalia, two decades of experience: clinical management and sex assignment

Ambiguous genitalia constitute a major social and medical emergency. This study was conducted to assess the relevant clinical significance of this important clinical entity. During the period 1989-2008, eighty-one patients with ambiguous genitalia were evaluated in a Pediatric Endocrine Clinic at King Khalid University Hospital [KKUH], Riyadh, Saudi Arabia. Of these 53 [65.4%] were genetically females [46XX], and 28 [34.6%] were having a male genetic sex [46XY]. The majority of them were proven to have congenital adrenal hyperplasia. Twenty-five [47.2%] of the genetic females were wrongly assigned a male sex due to severe virilization while only two [7.1%] of the genetic males were wrongly assigned as females. Although early neonatal diagnosis facilitates appropriate management, socio- cultural factors such as a bias concerning the male gender in the community and strong influence of the grandparents constitute major management obstacle. All genetic males who were wrongly assigned as females accepted re-assignment, and four [16%] of the 25 genetic females who were wrongly assigned as males refused re-assignment. A team approach is mandatory for successful management. Guidelines for approaching the problems are also highlighted

Homozygosity for the Mediterranean alpha-thalassemic deletion [hemoglobin Baits hydrops fetalis]

Hemoglobin Barts hydrops fetalis syndrome is the most severe and generally fatal clinical phenotype of alpha-thalassemia. We diagnosed a fetus at 23-weeks gestation with having hydrops fetalis, by ultrasound. At 32 weeks, intrauterine death was detected. Molecular studies revealed that the fetus had the hemoglobin Barts hydrops fetalis syndrome due to homozygosity for the Mediterranean alpha-thalassemia deletion. This clinical phenotype is generally rare in the Eastern Mediterranean, and this is the first report of this syndrome from Iraq. Techniques for molecular characterization became available only very recently in this country, in a diagnostic setting. Thus, the detection of further cases might be expected in future

Gastrointestinal duplications: experience in seven children and a review of the literature

Enteric duplication [ED] is a rare congenital anomaly that can occur anywhere along the alimentary tract from the mouth, down to the anus and the nearby organs. This uncommon anomaly may be asymptomatic or presents with vague symptoms mimicking other common pathologies. We aim to present our experience, management challenges and patterns of ED with a review of the literature. The study was carried out at a Nigerian Tertiary Hospital [2005-2008 inclusive]. We retrospectively analyzed seven patients with ED managed in our hospital for sex, age, clinical presentations, duplication size and site, presence of ectopic tissue, complications, associated anomalies, radiological workups, and prognosis. Data was analyzed using SPSS 11.0 for window. Seven children between the age range of 44 hours-10 years had ED, one sublingual and six intraabdominal duplications. Midgut volvulus with long segment bowel gangrene complicated two cases. The diagnosis was incidental in all. Three cases were diagnosed following conventional radiological contrast examination and the rest at surgery. Ultrasound was not helpful in making diagnosis in all the six intraabdominal duplications. Though surgery was recommended for all, one of the patients declined. Only one patient had unsuccessful surgery. ED requires high index of clinical suspicion and careful management. Many cases of nonspecific abdominal pains should be properly evaluated before patients suffer avoidable complications

Thrombophilic mutations in blood donors in Duhok-Iraq

Thrombophilia is a mulitfactorial disease due to the interplay between acquired and inherited factors. Factor V Leiden [G1691A], Prothrombin [G20210A] and MTHFR [C677T] are among the important inherited causes. The prevalence of these three thrombophilic mutations has not been addressed collectively in Iraqis, including the population of Duhok. Determine the prevalence of thrombophilic mutations among healthy blood donors form Duhok. One hundred and fifty random healthy blood donors from the regional blood bank in Duhok-Iraq were investigated using multiplex PCR and reverse hybridization to oligonucleotide specific probes to detect Factor V leiden and MTHFR C677T mutations. While the first hundred donors were also screened using the same technology for Prothrombin G20210A mutation. Factor V Leiden and Prothrombin G20210A carrier states were found in 1.25% and 3% of the individuals screened for them, respectively. The MTHFR C677T homozygous and heterozygous states were confirmed in 8 and 44% respectively. This study demonstrated that while the prevalence of Prothrombin and MTHFR mutations were rather consistent with pattern seen in surrounding countries in the Mediterranean region, Factor V Leiden prevalence was the least ever reported from any other population in the region. The latter finding suggests that the contribution of Factor V leiden to thrombotic states in Northern Iraq may not be as significant as it is in other countries in the region

Pediatric day case surgery: Experience from a tertiary health institution in Nigeria

Background : The general observation that children achieve better convalescence in the home environment supports the need for adoption of day case surgery, which is gaining considerable acceptance in developing countries. Pediatric surgical service is in great demand in developing countries, and in-patient beds and surgical supplies are insufficient. Method : A prospective collection of data on all pediatric day surgeries (PDSs) by the pediatric surgical unit University of Ilorin Teaching Hospital (UITH, Ilorin, was done. Parents had pre-operative outpatient briefing and postoperative interviews on the second and ninth days for consultation regarding post-operative complications and events at home. Study period was between April 2005 and September 2007 (2½ years). Results : Of the 660 elective cases, 449 (68.02%) children were recruited as day cases. The male-to-female ratio was 14.3:1. Age ranged between 20 days and 15 years with a mean of 37.6 months and standard deviation (SD) of 34.4 months. Congenital hernias/ hydroceles were the highest indications (71.2%), followed by lump/ masses (12.9%), undescended testes (8.7%), umbilical hernias (4.8%) and thyroglossal duct cyst (2.5%). In 98.9% of cases, the parents resided within 20 km radius of the hospital, and 91.5% of them could reach the hospital within 1 hour. Fathers and mothers of 80.1% and 77.1% of children, respectively, had above-primary education. More than half of the fathers (55%) were civil servants, while 30% were self-employed. The mothers were civil servants in 37.3% of cases, and 34% were self-employed. The average number of outpatient clinic visits before surgery was 2-3 visits (41.2%) with mean interval to surgery of 4-5 weeks (60.3%). Logistics (investigations and availability of operation list) and patient's fitness for surgery were statistically significant delay factors (P= 0.001). Conclusion : Parents reported 14 children to be irritable at home due to pain, while the others reported satisfactory day case experience. No unplanned admission or mortality was recorded, and only 3 (0.8%) parents would not recommend day case surgery to other people. Conclusion: Pediatric day case surgery is feasible for well-selected and monitored cases in our environment. Term neonates with informed parents are suitable for pediatric day case surgery. There is a need for a day case center to reduce waiting list at UITH

Bowel resection in Nigerian children

Background : Although bowel resections are commonly done for congenital malformations in children in developed countries; they usually follow neglected and preventable acquired diseases of the intestine in developing countries. Objectives : To determine the indications and outcome of bowel resections in children of a developing country in a university teaching hospital. Materials and Methods: Data of the patients operated (from birth to 15 years) was retrospectively collected over eight years (January 1999 to December 2006). The biodata of children included the following: Indications for operation; type of operations; duration of admission; and outcome of treatment including complications. Patients with Hirschsprung's disease were excluded from the study because bowel resection forms part of their definitive surgical management. Results : There were 70 patients (38 boys and 32 girls). The age ranged between four hours to 15 years (median; five months). There were 16 (22.9) neonates; 26 (37.1) infants; and 28 (40) grown children. The indications were congenital anomalies in the 16 neonates. Also; 23 (88.5) infants had intussusception; 2 (7.7) had midgut vovulusm and 1 (3.8) had congenital small intestine band. Among the grown children; typhoid ileal perforation (TIP) was seen in 14 (50.0); intussusception in 5 (17.9); and other causes in nine patients. Overall; intussusception was the most common indication for bowel resection; followed by TIP. A total of 24 patients developed 33 complications. Complications included wound infection in 47.8and anastomotic leak in 42.8. The duration of admission ranged between 4-35 days (median; 15 days). The overall mortality was 17.1-; which was highest among neonates (56.3); followed by the infants (26.9-). Conclusion : Bowel resections are mainly done for intussusception and complications of TIP at our centre. Late presentation; preexisting malnutrition; and nonavailability of parenteral nutrition contributed to unacceptable morbidity and mortality

Detection of anti-Haemonchus contortus antibodies in sheep by dot-ELISA with immunoaffinity purified fraction of ES antigen during prepatency.

Haemonchosis is a very common disease in small ruminants caused by H. contortus, a blood sucking parasite causing anaemia that may be fatal particularly to young animals. Therefore, detection of the infection during prepatent period is important for early treatment. Excretory-Secretory (ES) protein of H. contortus was purified through immunoaffinity chromatography. Dot -ELISA was performed with crude ES antigen as well as immunoaffinity purified fraction (F-1) with experimental and natural sera of sheep infected with H. contortus. Solid dot formation took place with 4 day, 1, 2 and 3 weeks post infection sera. Dot formation did not take place with negative control serum and uninfected control animal serum. When crude ES antigens was reacted to natural sheep sera having H. contortus infection, 60% sera samples showed solid dot formation whereas in F-1 fraction 75% of the sera samples showed solid dot indicating purified fraction was a more potent antigen. Crude ES and F-1 were also fractionated through SDS-PAGE. ES antigen revealed polypeptides in the range of 10 to 200 kDa of which 26, 32, 60 and 120 kDa were found more prominent. F-1 fraction on SDS-PAGE analysis revealed only four polypeptides of 26, 32, 60, and 120 of which 60 and 120 kDa were found to be most prominent. Results indicate that the purified fraction of ES antigen may be utilized for early diagnosis of haemonchosis. Further studies on cross antigenicity of this fraction with other nematode and trematode needs to be conducted.

Prevalence of haemoglobinopathies in sulaimani - Iraq

Thalassaemia major is an important health problem in Sulaimani, a large province at Northeastern Iraq, and the need to initiate a preventive program for this potentially fatal disorder is paramount. As a prerequisite to such program this study was to map the province for hemoglobinopathies. A total of 1472 subjects [736 couples] attending Sulaimani premarital Health centre were screened using red cell indices and sickling test. For those who MCV < 80 fI and/or MCH < 27 pg or had a positive sickling test, this was followed by Hemoglobin HPLC and iron studies. Based on above investigations, 61 individuals [4.14%] were found to have beta-thalassaemia minor, 4 [0.27%] sickle cell trait, 2 [0.14%] Hb C trait, and 2 [0.14%] delta beta-thalassaemia minor, and one [0.07%] had Hereditary Hemoglobin F Persistence [HPFH] homozygous state. Moreover, 49 individuals [3.3%] had alpha- thalassaemia, including one with Hb H disease [0.07%]. The study also revealed a consanguinity rate of 24.3% among the screened couples. The high prevalence rate of beta- thalassaemia carrier state and consanguinity, among premarital couples should further strengthen the need for initiating a preventive program for hemoglobinopathies in this region

Morbidity of colostomy closure in children

The care of colostomy remains a burden to the family while complications associated with its construction and closure can be tasking to the surgeon. The aim of this study was to evaluate the outcome of colostomy closure in children in our setting. A prospective review of 31 consecutive children undergoing colostomy closure in a 6 year period. There were 18 (58.1%) boys and 13 (41.9%) girls, aged 5 months 13 years (median 3 years). The median duration of colostomy was 16 months [range, 5 days -8 years]. Indications for colostomy were anorectal malformations 16(51.6%), Hirschsprung/'s disease 10(32.3%) and others 5 (16.1%). The site of colostomy was sigmoid colon 21, transverse colon 9 and caecum one. The type of colostomy was divided 18(58.1%) and loop12 (38.7%). Intraperitoneal and extraperitoneal closure was performed in 27(87.1%) and 4(12.9%) patients, respectively. Thirteen (41.9%) patients had 16 postoperative complications; postoperative pyrexia 4 (12.9%), prolonged ileus 4 (12.9%), surgical site infection 2 (6.5%), septicaemia 2 (6.5%). Post operative complication rate was significantly associated with the status of the surgeon (p < 0.05) and duration of surgery (p < 0.05). The median duration of hospital stay was 8 days [range: 5-35 days]. There was no significant difference in hospital stay in patients with postoperative complication and thosewithout (p > 0.05). Morbidity following colostomy closure can be high, particularly when the procedure is performed by a junior trainee