A case of late presentation of precocious puberty due to pituitary astrocytoma

The importance of assessing precocious puberty, especially in boys, is not only due to the great complications it has for the affected patients, but also to the fatal underlying diseases. Therefore, children with central precocious puberty should first undergo neuroimaging. In this case study, we present a 9.5-year-old boy who was referred to Rasoul-e-Akram Medical Center with increased intracranial pressure, nausea/vomiting, and severe headache having begun three months earlier. The development of secondary sexual changes had started two years earlier, and had been neglected. His testes, penis, and pubic hair were at the fourth Tanner stage. He had elevated luteinizing and follicle stimulating hormones. Microscopic evaluation confirmed low-grade pilocytic astrocytoma WHO grade 1. Emergency brain surgery was conducted in which the brain was decompressed, and chemotherapy was started postoperatively. Two years after the surgery, he remains under chemotherapy, with obvious sexual maturation and a height of 154 cm. Training families and medical staff efficiently can help prevent the late diagnosis and treatment of precocious puberty and, as a result, help patients in their social life. © 2015 Aktualn Neurol

Functional Abdominal Pain Syndrome in Morbidly Obese Patients Following Laparoscopic Gastric Bypass Surgery

Background: Roux-en-Y gastric bypass surgery (RYGBP) is one of the most common bariatric surgeries, which is being performed using various techniques like gastrojejunostomy by hand swen, linear or circular stapler. Abdominal pain is a common complaint following laparoscopic gastric bypass procedure (LGBP), which has different aetiologies, such as overeating, adhesion, internal herniation, bile reflux and many more. In this study LGBP was performed in an ante-colic ante-gastric pattern in a double loop manner and the prevalence and distribution of pain in morbidly obese patients undergoing LGBP was assessed. Objectives: The aim of this study was to analyze the distribution and frequency of post LGBP pain in morbidly obese patients. Patients and Methods: This study was performed on 190 morbidly obese patients referred to Hazrat Rasoul Hospital in Tehran. After LGBP, pain was measured in the following intervals: 24 hours, one week and one month after the operation. Before the operation onset, 2 mg Keflin and 5000 IU subcutaneous heparin were administered as prophylaxis. LGBP was performed using five ports including: one 11 mm port was placed 15-20 cm far from the xiphoid, one 12-mm port in mid-clavicular line at the level of camera port, one 5-mm port in subcostal area in ante-axillary region in the left, another 5-mm port in the right mid-clavicular area and a 5-mm port in sub-xyphoid. All operations were done by the same team. Staple was used for all anastomoses and hand sewn technique to close the staple insertion site. The mesenteric defect was left open and no effort was made to repair it. Results: The results of this study showed that 99.94 % of the patients had complains of pain in the first 24 hours of post operation, about 60% after one week and 29.5 % still had pain after one month. In addition, left upper quadrant (LUQ) was found to be the most prevalent site for the pain in 53.7% of the patients in the first 24 hours, 59.6% after one week and 16.8% after one month (except for obscure pain) with a significance of < 0.05. Conclusions: In this study, the authors analyzed the location and disturbance level of pain after LGBP, which could serve as a cornerstone for further researches. The authors suggest that long-term follow-up (for more than a year after operation) should be considered in future studies and also the relationship between the drainage site and pain should be investigated

Efficacy of a modified bier�s block in patients undergoing upper limb bone surgery

Background: Intravenous regional block, called the Bier's block, refers to an analgesic technique applied for soft tissue surgeries and closed bone manipulations of the limbs. There are a number of complications in traditional method of block, including pain in tourniquet site, immediate return of pain after tourniquet deflation, wound hemostasis and some others. Objectives: The aim of this study was to assess the outcomes and complications of our new method of blockage. Patients and Methods: In this experimental study, twenty-five patients undergoing hand surgery were prospectively studied. Induced anesthesia was a modifcation of the Bier's block with two concurrent changes including insertion of the intravenous cannula at the antecubital region rather than distal and the proximal anesthetic direction by an elastic band wrapped tightly around the proximal forearm distal to the cannulation site. The pain relief was measured by the verbal descriptive scale at intervals after block, during the operation, after deflation of the tourniquet and one hour after the operation. Results: This study showed the presence of analgesia at surgical and tourniquet sites during the operation in 96 of patients, as well as considerable pain relief at surgical site during one hour after deflation of the tourniquet. Conclusions: The study indicated advantages of this modified Bier's block compared to the traditional one including ability to perform surgery on upper limb bones and considerable pain relief at surgical and tourniquet sites during the operation until one hour thereafter. © 2015, Iranian Society of Regional Anesthesia and Pain Medicine (ISRAPM)

Adrenal insufficiency as a cause of loss of consciousness: A case study

Introduction: The current study introduces a case with adrenal insufficiency that was suspected to have congenital adrenal hypoplasia because of undescended testis and failure to thrive due to muscle involvement. Case Presentation: The patient was a 34-month-old boy who had loss of consciousness, hypotension, hypoglycemia, hyponatremia and hyperkalemia with severe failure to thrive, hyper pigmentation of mucosa, hypotonia and no hypospadias. He was resistant to serum therapy and responded to hydrocortisone. Laboratory study elucidated elevated levels of adrenocorticotropin (ACTH) and muscle enzymes, decreased serum cortisol levels. Discussion: Adrenal hypoplasia congenita is a differential diagnosis for adrenal insufficiency. Undescended testis, especially in absence of hypospadiasis, is a clue to diagnose and treat this lethal disease

Minimally invasive surgical interventions in the treatment of primary persistent hyperinsulinemic hypoglycemia of infancy

Hyperinsulinemia, diagnosed by laboratory tests, should be diagnosed and treated as soon as possible to prevent fatal complications such as neurological damage. Patients who are resistant to medical therapy should be treated surgically. Minimally invasive surgery, a newly developed approach, is a good choice among surgical procedures to avoid unnecessary extensive pancreatectomy. Here, a 12-year-old boy is presented with diagnosis of hyperinsulinemic hypoglycemia who had recurrent attacks of hypoglycemia and seizures from infancy. Because of his unresponsiveness to medical therapy and his family�s preference, he underwent laparoscopic pancreatectomy to reduce morbidity and hospital stay. Two years postsurgical follow-up revealed a normo-glycemic state. © 2015, Sociedade Brasileira de Endocrinologia e Metabologia. All rights reserved

Pre-operative rectal indomethacin for reduction of postoperative nausea and vomiting after laparoscopic cholecystectomy: a double-blind randomized clinical trial

OBJECTIVE: To evaluate the effect of pre-operative indomethacin suppository on postoperative nausea and vomiting in patients undergoing laparoscopic cholecystectomy. STUDY DESIGN: A double blind placebo-controlled randomized clinical trial. PLACE AND DURATION OF STUDY: Hazrat Rasoul Akram Hospital, Tehran, Iran, from February 2010 to September 2012. METHODOLOGY: One hundred and thirty patients, scheduled for laparoscopic cholecystectomy, were randomly divided into case and control groups. Sixty-five patients received indomethacin suppository and 70 patients received rectal placebo in the case and control groups respectively. All patients underwent the same protocol in laparoscopic surgery and anesthesia, then nausea and vomiting was recorded after 1, 6, 12 and 24 hours postoperatively and compared between the two groups. Independent-sample t test or Mann-Whitney tests were used for statistical analysis. Level of statistical significance was set at P ² 0.05. RESULTS: Patients' nausea was statistically lower in the case group at the 1st hour (43.1 vs. 92.9), 6th hour (20.0 vs. 68.6) and 12th hour (7.7 vs. 24.3) after surgery (for all periods, P < 0.001). Fewer patients in the case group experienced vomiting at the first (13.8 vs. 51.4) and 6th hour (0 vs. 20) after surgery (for both P < 0.001). The use of pethidine was also statistically less in the case group in the same hours after surgery (for all of them, P < 0.001). CONCLUSION: Rectal indomethacin before laparoscopic cholecystectomy led to lower postoperative nausea and vomiting

First report of inherited thyroxine-binding globulin deficiency in Iran caused by a known de novo mutation in SERPINA7

Background Thyroxine-binding globulin (TBG) is the main transporter of thyroid hormones in human serum, encoded by the gene TBG (SERPINA7), located in long arm of X-chromosome (Xq21-q22). Deficiency of SERPINA7 (serum protease inhibitor, clade A alpha-1 antiproteinase, antitrypsin, member 7) leads to inherited TBG deficiency. Several mutations have been reported in the coding and noncoding regions of SERPINA7 in association with TGB deficiency. Methods Automated chemiluminescence immunoassays were used to determine TSH, free and total T4 and T3 (fT4, TT4, TT3) and TBG. Direct DNA sequencing identified the mutation in SERPINA7. Results We present a 3 and 4/12 year old boy, born premature, who was mismanaged as hypothyroidism before referral to our center, and was diagnosed with TBG deficiency at our center with a hemizygous substitution in exon 1, position c.347T > A, leading to replacement of isoleucine for arginine in position 96 (considering the first 20 amino acid signal peptide). Conclusion This known mutation, reported as the first SERPINA7 mutation in Iran, emphasizes the point that endocrinologists should pay more attention to inherited TBG to prevent unnecessary treatment

Configuring a better estimation of kidney size in obese children and adolescents

Background: Obesity ignites numerous health and psychosocial problems and is associated with various comorbidities. Body mass index (BMI) is also independently associated with improved risk for numerous kidney disorders. As renal length is considered a vital parameter in the clinical assessment of renal patients, normal renal length has to be defined in accordance to BMI. Objectives: The aim of this study was to define normal kidney length in obese children, comparing ultrasound measurements of the kidney length in obese and non-obese children and adolescents, in order to reduce unnecessary evaluations for nephromegaly. Patients and Methods: Fifty obese children and adolescents and 50 non-obese children and adolescents, aged 1-19 years, were selected from patients of pediatric clinics in two hospitals (Rasoul-e-Akram and Shahid Fahmideh) in Tehran between June 2010 and 2012. After the nephrologist�s and endocrinologist�s approval, the largest longitudinal renal dimension was measured in deep inspiration position by abdomino-pelvic ultrasonography in both groups. Results: It was revealed that both kidneys in obese group were significantly larger than in control group (P = 0.044 and 0.040, respectively). Obesity status, height and age were proven to be significant and independent predictors of length of both kidneys. In both groups length of left kidney was significantly larger than that of right kidney (P < 0.001). Conclusions: A specific standard cut-point limit or norm gram has to be formulated for obese children and adolescents in order to facilitate the diagnosis of kidney diseases, including organomegaly, in these patients. © 2016, Growth & Development Research Center

Comparison between marital satisfaction and self-esteem before and after bariatric surgery in patients with obesity

Background: Obesity is one of the most common chronic diseases with important medical effects, as well as mental and social health problems. Bariatric surgery is one of the most effective treatments of morbid obesity. Objectives: Because of the possible psychological changes, and its effects on weight loss after surgery, the current study aimed to compare marital satisfaction and self-confidence in patients with obesity before and after bariatric surgery in Rasoul-e-Akram hospital in 2013. Materials and Methods: This prospective observational study was conducted on 69 candidates for bariatric surgery. Marital satisfaction and self-confidence were assessed before and six months after the surgery by Enrich marital satisfaction scale and Coopersmith self-esteem inventory. Descriptive statistics and T-tests were utilized to analyze data. Values of P � 0.01 were considered statistically significant. Results: Despite the improvement of sexual relationship, marital satisfaction scores significantly decreased from 141.26 ± 12.75 to 139.42 ± 12.52 six months after the surgery (P = 0.002). Satisfaction in scales of conflict resolution and communication showed a descending pattern (P < 0.001). No significant difference was found between self-esteem before and after the surgery (P = 0.321). Conclusions: Weight loss after bariatric surgery did not improve self-esteem and marital satisfaction six months post operatively; therefore, psychiatric assessment of patients before and after the surgery is crucial; since even if they are not associated with prognosis of the surgery, it is important to provide treatment for psychiatric problems. Prospective studies are recommended to assess post-operative changes of other psychological aspects. © 2016, Mazandaran University of Medical Sciences

The nomogram of penile length and circumference in Iranian term and preterm neonates

Background and objectives: The normal length of penis in preterm and term neonates is different among different nations, and is affected by various factors. The present study aimed to determine stretched penile length (SPL) values and cutoff level of micropenis in term and preterm Iranian neonates, for the first time. Materials and methods: All male neonates born in two general hospitals of Tehran (Akbarabadi, and Rasoul hospitals), center of Iran, with gestational age of 28-42 weeks were included and their SPL and penile circumference (PC) were examined on the first 3 days after birth by the same physician. Birth weight (BW), and height, gestational age, type of delivery, mother's age, twin/multiple pregnancy, drug, and medical history of mother during pregnancy were recorded and cutoff levels of two variables were calculated based on the collected variables for different gestational ages. Results: Among a total of 587 neonates, 203 neonates were born term and 384 preterm. Mean ± SD of neonates' BW were 2,682.51 ± 739.30 (850-4800) gr. Mean ± SD of their SPL was 22.48 ± 3.34 mm; 25.92 ± 1.54 mm in term and 20.66 ± 2.50 mm in preterm infants (P = 0.001). Mean ± SD of PC was 6.71 ± 1.31 mm; 8.14 ± 0.48 in term and 5.96 ± 0.92 in preterm infants (P = 0.001). SPL and PC were significantly correlated with type of delivery, number of parity, gestational age, BW, and crown-heel length, head circumference (P < 0.001). Conclusion: This study suggested that SPL in male neonates was 22.48 mm and PC was 6.71 mm, both correlated with gestational age and BW. Due to the ethnical variety of this cutoff points and lack of an appropriate study in Iran, these cutoff points can be used by all physicians as a reference for Iranian newborns (term and preterm), in order to prevent misdiagnosis of micropenis and genital disorders. © 2018 Soheilipour, Rohani, Dehkordi, Isa Tafreshi, Mohagheghi, Zaheriani, Jesmi and Salehiniya