Ovarian cyst enlargement in a 14 year old female with persistent ascities, severe hypothyroidism and elevated serum CA-125 level

A 14 year old female complained of abdominal pain and distention with vomiting. The physical exam showed thyroid enlargement and ascites. The imaging evaluation demonstrated a large ovarian cyst. Laboratory tests depicted hypothyroidism and marked elevation of Carbohydrate antigen 125 [CA-125] levels. As the bone age was 10 years, more retarded than the chronological age, Van Wyk and Grumbach syndrome was suspected. Treatment with thyroid hormone was initiated and the condition improved dramatically with disappearance of symptoms and signs 5 weeks later

Response surface methodology approach to the optimization of oil hydrocarbon polluted soil remediation using enhanced soil washing

One of the environmental concerns in recent decades is the prevalence of different pollutants in soil. Hence, the importance of remediation has led to the development of various methods to remediate polluted soil. Among these methods, soil washing has gained significant attention to treat polluted soils. In this paper, the response surface methodology was applied in order to determine the optimal conditions for total petroleum hydrocarbon remediation using nonionic surfactant Brij35 in soil environment. The effect of different factors in soil washing process including surfactant solution concentration and volume, washing time, age of pollution and frequency of washing are evaluated. The predicted values for total petroleum hydrocarbon remediation efficiency by the response functions are in a very close agreement with experimental data [R[-2] = 98.75%]. The second order model was developed as experimented response and optimal conditions were obtained by analyzing the contour and surface plots and also by solving the regression equation using LINGO 9.0 software. The optimal concentrations [8 g/L], volume of surfactant solution [500 mL], washing time [75 min], age of pollution [29 days] and frequency of washing [three times] are determined. In this optimal condition, the removal efficiency has been observed to be 93.54% which conforms to the results of process optimization using response surface methodology. Based on the results, it can be concluded that the response surface methodology is a suitable approach to determine the optimal conditions of soil washing to remediate organic hydrophobic pollutants using the nonionic surfactant Brij35 from the soil

overview to the structure and function of nuclear matrix

Although the genome is defined by its primary sequence, its functional properties, are determined by far more complex mechanisms and depend on multiple layers of nuclear organization. The architecture of the nucleus includes two overlapping structures: the chromatin and a framework structure named the nuclear matrix. Ultra-structural studies reveal that the nucleat matrix is a network consisting of branched core filaments masked with a large number of hnRNPs and regulatory proteins. This scaffold has b'een demonstrated to be an active and dynamic structure, anchoring the nuclear processes such as replication, transcription and splicing making nuclear domainslfoci. It is postulated that the nuclear matrix serves as a dynamic support to bring together specific DNA sequences with factors involved in the regulation of genome functions. In this review, we attempt to introduce the structurefand function of nuclear matrix as an active intra-nuclear factor, having a critical dynamic role to organize different nuclear functions. Studying in vivo variations of this epigenetic parameter has been suggested to all investigators interested in the field of chromatin structure and itsdynamics

Outcome of prosthetic thigh vascular access graft in hemodialysis patients: a prospective six year study

Arteriovenous fistulae traditionally have been placed in the upper extremity. Experience with groin hemodialysis access has been discouraging because of high infection rates and associated limb amputation. The aim of this study was to determine infection rates, patency rates, and possible predictive factors for prosthetic thigh angioaccess outcomes in our hemodialysis patient population. A prospective study was performed for 53 patients who underwent placement of thigh vascular access graft at Sina Hospital, Tehran University of Medical Sciences, between Jan 2000 and July 2005. Demographics, complications, and subsequent treatment were recorded. Survival distributions were plotted using the Kaplan-Meier method for graft survival [primary patency]. Log rank tests were used to evaluate for statistical differences in survival distribution between different groups. The primary patency rates were, 78.4%, 61.2%, 56.2%, and 50% at 3, 6, 9 and 12 months, respectively. There were 15 [28.3%] access failures, related to infection in 2 cases [3.7%], thrombosis in 10 cases [18.9%] and bleeding in 3 case [5.7%].There was no limb ischemia.No significant differences in infection rate or graft patency rates were found by patients' age and gender. A PTFE vascular access in the thigh is not associated with higher morbidity compared with the upper extremity, and should be considered as a promising alternative when upper extremity arteriovenous fistulas cannot be constructed .The incidence of infection and thrombosis in our series is comparable with rates reported in the literature for lower extremity polytetrafluoroethylene angioaccess grafts

[Etiological profile of short stature in a referral endocrinology clinic]

Physiological growth is a sensitive long-term indicator of child health. Impaired growth of children may be the first manifestation of a serious chronic disease. Short stature is a common pediatric endocrine problem. The short stature, although not a disease per se, is a manifestation of several diseases. Its early diagnosis and treatment is most of the time rewarding. Children between the age group of 2 to 15 years with growth retardation attending endocrine clinic in Loghman Hospital, Tehran, in October 2003 through October 2005were evaluated for short stature. Evaluation included: detailed medical history, physical examination and laboratory tests such as blood count, thyroid function, growth hormone screening, bone age estimation, and karyotypes. Centers for Disease Control [CDC] growth charts was used for percentiles. 188 children [110 boys, 78 girls] with growth retardation, whose heights were below 2 standard deviation score for age and gender, attended endocrine clinic. Normal variations accounted for 85.5% of all etiologies for reasons such as constitutional delay 49% and familial short stature 26.5% and a combination of both 9%. The rest [14.5%] consisted of pathological short stature. Growth hormone deficiency and hypothyroidism were the most common causes of pathological short stature. The most common cause of short stature was a normal variance followed by short stature caused by endocrine disturbances. In both sexes constitutional growth delay followed by familial short stature counted to the most common non-endocrine causes of short stature

[Duplication of appendiceals: a case report]

Background: appendiceal duplication is one of the rare appendiceal anomalies with an incidence between 0.004% and 0.009% appendectomy specimens. Herein we present a 34 year old woman with appendiceal duplication, which caused in acute abdomen

Case presentation: a 34-year-old woman was admitted on an emergency basiswith a 10-day abdominal pain, loss of appetite,nausea, and.There was localized tenderness at McBurney'spoint, moderate rebound tenderness, guardingThe blood count showed leukocytosis [WBC :16800/mm3] with a shift to the left.Appendectomy was done and patient discharge after 3 days. Because fecal fistula and limited response to conservative management right hemicolectomy was done and pathology report a second appendix in iliocecal junction

Conclusion: in conclusion, appendiceal duplications are a rare finding. They may be confused with cecal diverticular disease or even colorectal cancer. The classification system of these abnormalities is important as a missed Type B appendix may result in serious clinical and medico-legal consequences. In addition, a double appendix can be associated with other congenital abnormalities. It is therefore important that trainee surgeons are aware of the anatomical anomalies and malpositions of the appendix

[Prediction of early failure of vascular-access arteriovenous fistula based on immediate postsurgical evaluation]

For patients requiring chronic hemodialysis, the preferred site for vascular access is an autogenous arteriovenous fistula. Although a properly formed fistula is advantageous because it is less susceptible than other types of vascular accesses to infection and clot formation and can last longer than any other types of vascular access, AV fistula has a high rate of early failure that can increase immediate cost and complications. In this study, the prognostic value of physical examination of arteriovenous fistula by the surgeon at the end of the surgery was evaluated. In the general surgery ward of Imam Khomeini Hospital in 326 chronic renal failure patients, 354 arteriovenous fistula operations were accomplished by two surgeons from 1377 to 1381 [ca. 1998 to 2002]. The performance of each fistula was divided into the following groups by the surgeon at the end of operation: 1] systolic and diastolic thrill, 2] systolic thrill 3] souffle 4] pulse 5] not functional. Clinical function of the fistula was evaluated by the same surgeon in the following days if no souffle or thrill, early failure was detected on initial inspection.: In the 354 cases of arteriovenous fistula, the total early failure rate was 12.7%. The lowest early-failure rate was 3.5% in the systolic and diastolic thrill group. The highest early-failure rate was in the not functional group [P<0.001]. There was no correlation between early failure and age, sex, surgeon and location of fistula. Optimally, an arteriovenous fistula has a thrill with a soft compressible pulse. At the end of each operation, if the surgeon cannot detect a thrill at the fistula site, can find only pulse, or if the function is otherwise unsatisfactory, considering of a new arteriovenous fistula may be required, however it is better to postpone the surgery

[Aorlobiiliac aneurism and ectopic kidney: a case report]

Arterial aneurysm is defined as a dilatation that exceeds 1.5 times the normal diameter. Abdominal Aortic Aneurysm [AAA] frequently involves infra-renal arteries. The common iliac and internal iliac vessels may also be involved. This disease usually involves males with a familial and sex-linked pattern. In this article, a case of abdominal aortic aneurysm [AAA] along with bilateral iliac arteries aneurysm and right ectopic kidney is reported. The patient was a 71-year-old man, smoker, who suffered from vague abdominal pains for 3 months without any changes in bowel habits or other symptoms. The pain was localized in mid-abdomen and upper abdomen, radiating to the back, and aggravating by physical exercise, but it would not change with feeding or position. In past medical history, hypertension was noticed for which the patient was on antihypertensive medications. Physical examination revealed a pulsating palpable mass in mid-abdomen. CT scanning showed dilation of abdominal aorta below the renal arteries, as well as bilateral iliac artery aneurysms. The right kidney was ectopically located in right pelvis and other abdominal organs were found normal

Effect of cAMP on induction of apoptosis in alveolar macrophages

cAMP as a second messenger and an intracellular regulatory agent has been considered to play a role in various biological processes. Some of compounds exert their effects via cAMP. In this study, the effect of cAMP on induction of apoptosis in alveolar macrophages was investigated. Alveolar macrophages were lavaged from rat lung and exposed to various concentrations of cAMP [0.5-200 micro M] and then they were incubated for 24 hours at standard condition. The effect of cAMP was investigated by measuring percent of viable cells, superoxide anion production and DNA fragmentation. The results show that low concentrations of cAMP [<50 micro M] enhance macrophage survival, but at higher concentrations [>100 micro M] a significant decrease in the cell survival is occurred. Analysis of anion superoxide production indicates no significant increase using low concentrations of cAMP [<50 micro M], but at higher concentrations of cAMP [>100 micro M] both superoxide anion production and DNA fragmentation could be increased. The results show that the effect profile of cAMP is dose dependent. So low concentrations of cAMP prevent apoptosis of macrophages, whereas at higher concentrations induces apoptosis in the cells

Mandibuloacral dysplasia in an Iranian girl

Mandibuloacral dysplasia [MAD] is a rare autosomal recessive syndrome. Less than 25 families have been reported, most of which are Italian. Here, we describe a new patient of Iranian origin, born to consanguineous parents

[Exercise in type 1 diabetic patients]

Having sufficient daily activity has great physical, psychological and social impacts on children and adolescents' life. Diabetic youth are of no exception in this regard, but they are often exempted from sport activities and its benefits due to the general concerns about possible side effects of diabetes, like hypoglycemia. There has been a general unawareness about appropriate diet and tapering the medications with the level of physical activities. There are several influencing factors that may prevent or at least lessen these side effects. These may include, appropriate timing of the insulin administration and knowing the timing of its peak activity onset according to the type of insulin used. Also adjusting the carbohydrate intake with type and extent of exertion are of significant importance. Meanwhile, both diabetic athletes and their trainers must be educated about signs of hypoglycemia and how to manage it on the scene. Diabetic patients, whose disease hasn't been effectively controlled yet and suffer from poorly controlled blood sugar and side effects like diabetic ketoacidosis, must avoid any sport activities until they have reached to a steady metabolic state. When initiating a new exercise regimen, patients must check their blood sugar levels 3 to 4 times daily, until all the metabolic needs have been identified and met. Needless to say those diabetic patients who have previously adopted an inactive lifestyle must undergo a complete medical evaluation prior to engaging in any exercise routine. Those, who have no evidence of diabetes complications, may engage in any level of recreation or professional sports. As a matter of fact, aerobic sports are among those most recommended to diabetics. If the essential educations regarding diabetes be provided for both the diabetic adolescents and their coaches, sports may be introduced as a safe and invaluable experience in a supervised environment

Radiocontrast nephropathy: is it dose related or not?

To assess the safety of high dose non-ionic contrast media during a single radiological procedure in patients with pre-existing renal impairment. One hundred eighteen patients, with serum Creatinine greater than 1.3 mg/dl who were undergoing coronary angiography or percutaneous transluminal coronary angiography [PTCA] were included in the study. All patients received the nonionic dye ULTRAVIST [Iopromide]. Serum creatinine were measured before, 48 hours and 1 week after the administration of contrast agent. An acute contrast induced reduction in renal function was defined as an increase in Serum Creatinine concentration of >=0.5mg/dl, 48 hours after the administration of contrast agent. All patients with end stage renal disease or patients undergoing coronary bypass surgery within a week after coronary angiography or had any concomitant factors that could cause acute renal failure e.g., sepsis, hypotention, etc., were excluded. Patients receiving a dose of upto100 ml of contrast agent [low dose group] were separated from those who received greater than 100 ml of contrast agent [high dose group]. Patients in both groups had similar characteristics in terms of sex, age, weight and underlying disease. Student's t-test was used for statistical analysis. The mean age of our patients was 62.3 + 8.83 [range 40 - 84 years]. There were 93 [78.8%] males and 25 [21.2%] females. The mean pre-contrast creatinine in the low contrast group was 1.97+0.92 and high dose group was 2.16+1.90 [p=0.48]. The post-contrast Creatinine at 48 hours was 2.11+1.11 and 2.06+1.39 in the groups receiving low and high dose contrast agents respectively [p=0.830], while at 7 days post-contrast it was 2.17+1.28 and 1.95+1.43 respectively in the two groups [p=0.391]. The contrast-induced reduction in renal function [rise in serum Cr >=0.5 mg/dl above base line] occurred in 14% [n=8] of patients in low dose and in 11% [n=7] in high dose contrast group [p=0.830, insignificant]. The results of our study confirm that high dose non-ionic contrast is not associated with increased risk of contrast-mediated nephrotoxicity in patients with pre-existing renal insufficiency undergoing cardiac angiography [p=0.830, insignificant]

Maximum tolerable dose of cyclophosphamide and azathioprine in Pakistani patients with primary renal disease

Objective: The immunosuppressive regimens, at present, mainly rely on western guidelines that were derived from studies conducted in western populations. No such study exists for South Asian population, which is home to almost two billion people different in both genetics and environment from west. Locally derived thresholds for side effects markedly different from western figures may warrant re-adjustment of current local immunosuppressive regimens that are at present based largely on western guidelines. In order to define optimum dose for Cyclophosphamide [CYC] and Azathioprine [AZA] based immunosuppressive therapy, we conducted this study to find out maximum tolerable doses of azathioprine [AZA] and cyclophosphamide [CYC] beyond which neutropenia and thrombocytepenia are most likely to occur in patients with primary renal pathology

Method: Patients with systemic vasculitis and idiopathic glomerulonephritis who were on CYC and AZA were identified through review of medical records at a tertiary care hospital in Pakistan [The Aga Khan University Hospital, Karachi]. Patients were categorized under three principal diagnosis i.e. systemic lupus erythematosus [SLE], primary [idiopathic] glomerulonephritis [GN] and Wegener's granulomatosis [WG]. The Receiver Operating Curve [ROC] was used to calculate the maximum tolerable dose for both CYC and AZA

Results: We identified 94 patients aged 6-82 years [median 44.5 years] with primary renal disease [Wegener's granulomatosis n=13, Systemic lupus erythematosis n=62 and idiopathic glomerulonephritis n=19] who received CYC or AZA. Of these 94 patients, 36.2% [n=34] received CYC and 63.8% [n=60] received AZA. The mean dose of CYC was 1.54 +/- 0.50 mg/kg of body weight [range: 0.77-2.93]. The mean dose of AZA was 1.64 +/- 0.59 mg/kg of body weight [range: 0.47-2.97]. The maximum tolerable doses calculated for CYC and AZA were 1.25 mg/kg and 1.30 mg/kg of body weight respectively. The maximum tolerable dose for CYC and AZA among males could not be calculated, because of insufficient number of patients who developed neutropenia and thrombocytopenia. The maximum tolerable doses for CYC and AZA among females were 1.34 mg/kg and 1.03 mg/kg of body weight respectively. Also we found out that AZA was relatively more likely to cause neutropenia and thrombocytopenia [p = 0.07]

Conclusion: We thereby recommend that CYC should be initiated at a dose no more than 1 mg/kg of body weight and AZA at an initial dose of 0.75-1.0 mg/kg of body weight. The dose may be adjusted later on the basis of clinical response and laboratory reports

Cutaneous maingestations of systemic lupus erythematosus in pakistani patients

Systemic Lupus Erythematosus [SLE] is an autoimmune process in which cutaneous lesions occur in majority of patients. This study from Karachi, Pakistan was conducted to determine the pattern and prevalence of such lesions in SLE in Pakistani patients. One hundred ninety eight patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association were examined between 1986 and 2001' for the presence of cutaneous manifestations. Skin changes noted were: noncicatricial diffuse alopecia [22%], malar rash [31%], mucosal lesions [20%], discoid eruptions [15%], photosensitivity [33%], vascular lesions [20%], pruritis [17%], and pigmentary changes [22%]. Peripheral gangrene,chronic ulcers, Raynauds phenomenon, urticaria, chilblains, thrombophlebitis, palmar erythema, and erythema multiform were rare. Anti ANA and anti dsDNA were positive in 93% and 83% patients respectively. A different clinical pattern was noted in our patients than reported previously

Spetrum of complications and mortality of bacterial meningitis: an experience from a developing country

The aim of this study was to obtain data on predisposing factors, causative organisms and their associated mortality and complications related to acute bacterial meningitis. The chart review of all patients in whom acute bacterial meningitis was diagnosed at The Aga Khan University Hospital from January 1995 through December 2001. One hundred ninety-four patients were included in study. There were 146 males and 48 females. The mean age of our study population was 41 +/- 12.3 years. One hundred and ninety [97.9%] patients had communityacquired meningitis-, only 4 [2.0%] patients developed meningitis nosocomially. The two most common predisposing factors were diabetes mellitus [13.9%] and otitis media [7.7%] among all 194 patients. A significant proportion of patients with complications had diabetes mellitus [24.6%, p<0.001]. CSF and blood cultures were positive in 53 [27.3%] and 42 [21.6%] patients respectively-, there was no statistical difference found. The most common organisms isolated were Streptococcus pneumoniae in 35 [36.8%] patients followed by Neisseria meningitides in -30 [31.5%] -patients. Approximately 68% of -positive cultures -yielded S. pneumoniae and N. meningitides [p<0.0001]. The overall mortality rate was 22.1%. The mortality rate for Streptococcus pneumoniae was 17.1%. The highest mortality was observed in patients with Pseudomonal meningitis where all four patients expired followed by mortality rate of 85.7% in Escherichia coli afflicted patients [p<0.001]. Complications occurred in 73 [37.6%] patients with persistent complications in 31 [42.4%] patients. Complications resolved in 34 [46.5%] patients. The most common complications were seizures [12.8%] and cranial nerve palsies [11.3%]. Seizures were more likely to occur in older patients [p<0.05] whereas hydrocephalus was more common in younger patients [p<0.05]. Bacterial Meningitis remains a serious disease associated with substantial morbidity and mortality. Most cases are community acquired with S. Pneumoniae being the most common pathogen. Old age, diabetes mellitus, a positive culture, seizures as a complication and late stage in the disease are the important predictors of a poor outcome

Report of 285 patients with congenital adrenal hyperplasia and evaluation of approximate prevalence of the disease in Iran

In this study, 285 cases of congenital adrenal hyperplasia who were followed in the Tehran University Hospitals and Institute of Endocrinology and Metabolism are reported. Among these cases, 165 [57.9%] were female and 120 [42.1%], male. The most common type of congenital adrenal hyperplasia in these patients was the salt-losing type of 21-hydroxylase deficiency [57.9%]; 11-hydroxylase deficiency was present in 13.68% of patients. There were only 3 cases with 3-beta hydroxysteroid dehydrogenase deficiency, 2 cases with 17-alphahydroxylase deficiency and one with 20, 22 desmolase deficiency. Presenting complaints were in decreasing order of frequency: ambiguous genitalia, vomiting and dehydration, precocious puberty, hypertension, failure to thrive, hirsutism and primary amenorrhea. The age of patients at the time of diagnosis was between 2 days to 17 years and the most common age was in the first two years of life especially in the neonatal period. A positive family history of the same disease was present in 17 siblings of our patients. [21-OHD = 14 H-OHD = 3]. There were 27 cases of death among these patients [23 male and 4 female that 24 cases had 21-OHD and 2 cases had 3 beta HSD deficiency and one case had 20,22-desmolase deficiency]

Evaluation of clonidine, insulin, L-Dopa, exercise tests on growth hormone in short children

Growth hormone stimulation tests have been used to assess the growth hormone reserve of the pituitary gland in both children and adults. We have assessed the effect of clonidine, insulin, L-Dopa and exercise on growth hormone secretion in 261 short children. The results found in this study revealed that there are no significant differences in these stimulation tests [P=0.28]

Tetanus- a community health problem in Hazara division

Prevalence, pattern, and prognosis of tetanus was studied in 100 admitted patients in Civil Teaching Hospital, Abbottabad between January 1992 to October, 1995. Tetanus was found to be more common in men and was more prevalent in summer. Survival of our tetanus patients was comparable with various centres equipped with intensive therapeutic supports, Prevention campaign need to be enforced to enhance mainly status of the community

effect of theophylline on the kinetics of cAMP-dependent protein kinase catalytic subunit, cAMP, Protein kinase inhibitor and their relationship in lung tissue

We have investigated the effect of theophylline on the kinetics of the catalytic subunit of protein kinase and related factors in lung tissue. The results show that the point of highest concentration of the C subunit of protein kinase which is active in casein phosphorylation is at 3h of incubation time, but in the presence of 100 micro g/ mL and 10micro g/mL the ophylline, this is shifted to 1.5 and 2.5 hrs, respectively. Also the maximum concentration of cAMP for the control is at 2.5 h of incubation time, but in the treated samples shifts to 2.15 and 1.15 hrs, respectively. Inhibitor protein content also changes considerably in the presence of 10micro g/mL theophylline. The results suggest an effect of theophylline on the function of cAMP-dependent protein kinase

Feedback regulation of colony stimulating factor production

The production of colony-stimulating factors [CSF] is delicately controlled through a complex network of humoral and environmental factors. We have studied some of the mechanisms which regulate the production of CSF as compared to general protein synthesis in the lung tissue in vitro. When lung tissue from mice was cultured for various times in serum free medium, the first detectable level of CSF activity in the lung conditioned medium [LCM] appeared 6 hr after initiation of the culture, continued to rise until 24 hr, and then levelled off for several days. Under similar conditions protein synthesis did not level off, but continuously rose after 24 hr. When the lung tissue which had been cultured previously for 6,24, or 48 hr was recultured in fresh tissue culture medium, de novo synthesis of CSF occurred as judged by CSF synthesis inhibition and stimulation studies. The amount of new CSF synthesized by these tissues decreased as the initial culturing period increased from 6 to 48 hr. There was also a decrease in the amount of total protein synthesis and release in the secondary lung cultures as a function of the initial culturing period. Endotoxin stimulation of 24 or 48 hr cultured lung tissues [plateau phase tissue] resulted in de novo synthesis of CSF by these tissues. However, when fresh lung tissue was cultured in 24or 48 hr LCM, no new CSF was produced by the fresh tissues, while under similar conditions, protein synthesis by these tissues was significant as judged by double-labelling experiments. On the other hand, 6 hr LCM was able to support both CSF production and protein synthesis by fresh lung tissues. The results suggest at least two distinct regulatory systems controlling CSF production by the lung in vitro: 1- Aging which is responsible for general and nonspecific decrease in the rate of protein synthesis and CSF production in this system, and 2-Feedback regulation of CSF production by the level of CSF which is formed in the LCM