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Congenital adrenal hyperplasia comprises a group of disorders resulting from defects in enzymes required for the synthesis of cortisol. The clinical presentation depends on the specific enzyme defect. We report a rare case of congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency. A 26-year-old female patient referred with hypertension and hypokalemia. She also had primary amenorrhea and lack of sexual development. The karyotype was 46, XY. Hormonal evaluation showed low serum levels of all steroid hormones, requiring alpha-hydroxylation, which included cortisol, 17 alpha-hydroxy progesterone, dehydroepiandrosterone sulfate, estradiol, and testosterone. The levels of adrenocorticotropic, follicle-stimulating, and luteinizing hormones were high. Radiological and surgical investigations failed to show a gonad. She also had a large myelolipoma. Treatment was commenced with low-dose dexamethasone and conjugated estrogen. Her hypertension and hypokalemia were resolved. The myelolipoma was removed by laparoscopy due to pain and sensation of heaviness. Our review of literature revealed that a combination of this disorder with either agonadism or myelolipoma is very rare and that only 2 previous cases have been reported for each entity.

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Acute rejection is a common phenomenon in transplantation. Inflammatory and anti-inflammatory mediators affect the graft microenvironment. Th1 responses cause acute rejection while Th2 immune responses help the survival of the graft. In this study, we evaluated gene polymorphisms of IL-6 G-174C, TGF-ß T+869C, IL-4 C-590T, and IFN-γ T+874A cytokines in renal transplant patients. ARMS-PCR method was used to characterize IL-6 G-174C (rs76144090), TGF-ß T+869C (rs1800471), and IFN-γ T+874A (rs2430561) polymorphisms and PCR-RFLP, for characterization of IL-4 C-590T (rs2243250) in 100 renal transplant patients. Acute rejection episodes were diagnosed according to the standard criteria. Analysis of the results showed that IL-6-174 GG genotype (P=0.018, OR=3.023, 95% CI=1.183-7.726) and IL-6-174G allele (P=0.046, OR=2.114, 95% CI=1.005-4.447) were more frequent, but IL-6-174GC genotype was less frequent in acute rejection of kidney transplantation in comparison with control group (P=0.024, OR=0.302, 95% CI=0.103-0.883). IFN-γ+874 T allele was associated with a higher risk of acute rejection (P=0.019, OR=2.088, 95% CI=1.124-3.880) while IFN-γ+874 AA genotype was associated with a lower risk of rejection (P=0.023, OR=0.318, 95% CI=0.115-0.875). Frequencies of TGF-ß T+869C and IL-4 C-590T were not significantly different (P>0.05). Consequently, our results show that IL-6 G-174C and IFN-γ T+874A gene polymorphisms have predictive values for acute rejection after renal transplantation in Iranian patients.

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BACKGROUND: Renal dysfunction (RD) is a common complication following liver transplantation. Postoperative renal function after split liver transplant (SLT) and (partial living related liver transplant) (LRLT) has not been well studied yet. MATERIAL/METHODS: Renal function immediately after surgery was analyzed retrospectively in 32 patients that received SLT and LRLT. Serum creatinine (SCr) was measured before surgery, and, after transplantation daily during the first week and at 14, 21, and 28 days after transplantation. Patient's medical records were reviewed to find clinical data;Model for end-stage liver disease (MELD) score, Child-Turcotte-Pugh score (CTP) class, the length of surgery, length of anhepatic phase, hospital and ICU admission, incidence of acute rejection, renal dysfunction, and sepsis. These data compared between groups. RESULTS: Length of surgery and anhepatic phase was longer in SLT and LRLT group (P<0.05). The incidence of acute rejection, reoperation, and complication such as sepsis was higher in SLT and LRLT group than FSLT group (P<0.05). There were no significant difference between groups with respect to MELD, CTP score, the need for transfusions, the length of admission to the hospital and ICU. Immunosuppression regimens were similar in both groups. RD developed in 25.8% of SLT and LRLT patients, but in only 9.5% of FSLT patients (p=0.063). The requirement for RRT in SLT and LRLT group (12.5%) was greater than that in the FSLT group (2.3%); P=0.20. CONCLUSIONS: Although the number of patients studied was small, our data suggests a higher incidence of RD in patients receiving SLT and LRLT.

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Management of renal transplant patients requires periodic measurement of renal function especially in early post transplant period. This is usually assessed by measuring the creatinine clearance, but because of its limitations, it is not an ideal marker for assessing the renal function. Serum cystatin C (sCyC) appears to be an endogenous marker of glomerular filtration rate (GFR). To assess the use of sCyC as a marker of renal function in kidney transplant patients, we compared it with serum creatinine (sCr) and 24-hour urine creatinine clearance (CrCl) in the first week post-transplantation. Among 60 patients (62.8% men, 37.2% women) undergoing kidney transplantation (average age: 44.87 +/- 13.37 years), we determined renal function at 1, 3, 5, and 7 days after kidney transplantation using: sCr, sCyC and CrCl in a 24-hours urine specimen. During the first 5 days following transplantation, there was a progressive decline in sCr levels. In the first 5 days, post transplantation we could not find good correlation between CrC and sCyC, and the sCyC increased during these 5 days, but after that in day 7, there was a good correlation between CrC and sCyC which is coinciding with decreasing the dose of steroid (r= .625). Therefore, we recommend using sCyC may be used as a marker of renal function after one-week post kidney transplantation.

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BACKGROUND: Pancreas transplantation is the treatment of choice for selected patients with type 1 diabetes mellitus. We reviewed our first 40 patients who underwent pancreas transplantation in Shiraz Organ Transplant Center. METHODS: Between April 2006 and April 2008, we performed pancreas transplantation on 40 recipients. The operation included portal venous drainage and exocrine enteric drainage. Immunosuppressive therapy included prednisolone, tacrolimus, and mycophenolate mofetil. Ganciclovir was administered as prophylaxis for cytomegalovirus. Peri-operative and regular follow up data on survival and complication were gathered and analyzed. RESULTS: The mean follow-up was 10.1+/-6.5 months (range: 1 - 24 months). Mean age of donors and recipients was 23.6+/-8.2 and 32.30+/-8.9 years, respectively. The mean pretransplant insulin consumption was 43.75+/-17.4 IU. Fasting blood glucose before transplantation was 275.5+/-72.3 mg/dL that decreased to 95.6+/-7.01 at six months follow-up (P<0.001). Complications were as follows: re-exploration (n=9), gastrointestinal complications (n=10), acute rejection episodes (n=12), and chronic rejection (n=4). We lost one patient due to diffuse cytomegalovirus and aspergillus infection three months after the operation with a functioning graft. Overall graft survival was 84.9% and patient survival 97.5%. CONCLUSION: Good patient and graft survival in these series encouraged us to continue the program with all its difficulties.

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Data regarding the socioeconomic status in Iranian kidney transplant (KT) recipients is lacking. In this cross sectional descriptive study we evaluated the socio-economic status of 100 KT recipients in Shiraz organ transplantation center. In a cross-sectional design, we randomly selected and interviewed 100 RT recipients (50 males and 50 females). Data regarding age, gender, martial status, occupation, level of education, number of children, type of insurance, monthly household income, place of residence, ownership of a personal transportation device, duration and frequency of pre-transplant dialysis, family history of CRF (Chronic renal failure), and etiology of renal disease were obtained. There were 50 (50%) patients aged between 16 and 35 years, 55 had a family history of CRF, 60 had been on dialysis for more than a year, 61 were married, 47 did not have any children, 41 had more than 3 children, and 65 were unemployed due to physical and emotional impairment as a result of their disease. The majority (73%) did not have a high school diploma, 15% were illiterate, 85% were below the poverty line, 52% were from rural areas, and 98% were covered by insurance. We conclude that patients with CKD in our study had acquired this condition possibly due to negligence and lack of basic health care in the lower socioeconomic class. In addition, KT is an available therapeutic modality to lower socio-economic level in Iran.

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With increasing long-term graft survival and life-long immunosuppression, cardiovascular disease and infectious complications are major causes of morbidity and mortality. We retrospectively evaluated 1200 consecutive kidney transplant patients at Shiraz Organ Transplant Center from December 1988 to December 2003. Data on demographic profile, donor source, blood pressure, rejection episodes, cause of death, and hematological and biochemical serum profiles were collected to compare alive and dead recipients. One hundred fifty six patients (13%) died in the post transplant period. Patient death was more prominent during the first years after transplantation. Most common causes of death were cardiovascular (28.3%), graft loss (20.7%), and infection (19.6%). Post transplant systolic and diastolic blood pressures, BUN, creatinine, fasting blood sugar, and total cholesterol were higher, and serum HDL lower in the dead recipients than those who remained alive (P< 0.05). No significant difference was found in LDL, hemoglobin, and triglyceride values between the two groups. The one- and three-year patient survival rates were 94% and 91.5%, respectively. We conclude that the patients who died after transplantation had more risk factors than stable patients who remain alive. A multidisciplinary approach to control the co-morbid factors could be beneficial to decrease the mortality of patients after renal transplantation.

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Postoperative acute renal failure is a frequent and serious medical complication following orthotopic liver transplant. Here, we report our experiences with liver transplant recipients who developed acute renal failure in the early period following orthotopic liver transplant. Among 100 liver transplants performed between April 1993 and January 2004, we retrospectively analyzed 91 patients (mean age, 29.9 +/- 14.0 years) who had undergone orthotopic liver transplant. The underlying causes of liver failure were cryptogenic liver cirrhosis (n=27), viral hepatitis (n= 21) (hepatitis-B-related liver cirrhosis [n=13], hepatitis-C-related liver cirrhosis [n=7], and hepatitis-B- and C-related liver cirrhosis [n=1]), autoimmune hepatitis (n=18), Wilson's disease (n=10), primary sclerosing cholangitis (n=8), biliary atresia (n=3), Budd-Chiari syndrome (n=2), and primary biliary cirrhosis (n=2). The immunosuppressive regimen included mycophenolate mofetil (azathioprine for 10 patients), cyclosporine, and steroids. Six patients received a combination of tacrolimus and steroids. Ten patients (10.9%) experienced acute renal failure, 7 (70%) were men, and none of them required renal replacement therapy and/or died. Four patients were diagnosed as having cryptogenic liver cirrhosis; 2 with hepatitis-C-related liver cirrhosis, 2 with autoimmune liver cirrhosis; 1 with primary biliary cirrhosis; and 1 hepatitis-B-related liver cirrhosis. Six patients were Child-Pugh's classification C, and the others were B. The rate of postoperative acute renal failure in our patients was relatively low when compared with other series, and our outcomes were good.

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