Background: Imperforate anus with recto-vestibular fistula is the most common form of imperforate anus in females and is an intermediate type of this disease, which is treated surgically. The current study aimed to compare three techniques used for the treatment of imperforate anus with recto-vestibular fistula. Materials and Methods: Fifty-four cases of imperforate anus with recto-vestibular fistula were managed using three main approaches: posterior sagittal ano-rectoplasty, anterior sagittal ano-rectoplasty, and the anal transposition or trans-sphincteric ano-rectoplasty (TSARP). The functional results and cosmetic appearance for each case after treatment were evaluated in patients above 3 years of age (43 cases). Results: The functional outcomes were good in all treatment groups; however, the better cosmetic results were evident in the group treated using TSARP, a technique, which preserves the skin bridge between the new anus and the posterior fourchette, does not disturb the perineal body, and avoids division of the levator muscles. In this group, a continence rate of 100% but a higher constipation rate (41%) than in the other groups was recorded. Conclusion: TSARP is recommended for imperforate anus with recto-vestibular fistula.

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Growth hormone (GH) is a peptide hormone necessary for normal postnatal growth as well as human metabolism. It works via its widely expressed receptor (GHR), which is a dimer of transmembrane glycoproteins, belonging to cytokines type I receptor family. Signaling via GHR is initiated by conformational changes triggered upon GH association. GHR is recognized as an important therapeutic target for treatment of many pathologic conditions, including growth disorders and cancer by designing highly specific and efficient GHR agonists or antagonists. However, engineering of such molecules requires deep understanding of the structure and function of both GH and GHR protein molecules. This work reviews the current status of knowledge covering the molecular structure of both GH and GHR and their molecular interactions, in addition to different GH agonists or antagonists developed to regulate GH action.

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In benign prostatic hyperplasia (BPH), there will be a sudden impact on overall quality of life of the patient. This disease occurs normally at the age of 40 and above and is also associated with sexual dysfunction. Thus, there is a need to update our knowledge on the current medications for this disease. The presented review provides information on medications available for BPH. Phytotherapies with some improvements in BPH are also included. Relevant articles were identified through a search of the English language literature indexed on MEDLINE, PUBMED, ScienceDirect, and the proceedings of scientific meetings. The search terms were benign prostatic hyperplasia, BPH, medications for BPH, drugs for BPH, combination therapies for BPH, and phytotherapies for BPH. Medications including watchful waitings, β₁ -adrenoreceptor blockers, 5β-reductase inhibitors, and combination therapies including tamsulosin-dutasteride, doxazosin-finasteride, terazosin-finasteride, tolterodine-tamsulosin, and rofecoxib-finasteride were found. Herbal remedies such as Cernilton, Saxifraga stolonifera, Zi-Shen Pill (ZSPE), Orbignya speciosa, Phellodendron amurense, Ganoderma lucidum, Serenoa repens, pumpkin extract, and Lepidium meyenii (Red Maca) showing some improvement on BPH are included. Other than these, discussions on transurethral resection of prostate (TURP) and minimally invasive therapies (MITs) are also included. Recent advancements in terms of newly synthesized molecules are also discussed. Specific β₁ -adrenoreceptor blockers such as tamsulosin and alfuzosin will remain the preferred choice of urologists for symptom relief. Medications with combination therapies still need more investigation to establish their preference in the initial stage for fast symptom relief, reduced prostate growth, and obviously for reducing the need for BPH-related surgery. Due to lack of proper evidence, phytotherapies are not gaining much advantage. MITs and TURP are expensive and are rarely supported by healthcare systems.

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Crisponi syndrome is a rare autosomal recessive disorder caused by mutation in the Cytokine Receptor-Like Factor1 (CRLF1) gene. The cardinal features of this syndrome, which are always evident at birth, consist of muscular contractions of the facial muscles in response to tactile stimuli with trismus simulating tetanic spasm, abundant salivation, major feeding and respiratory difficulties, characteristics dysmorphic features, camptodactyly and hyperthermia. In infancy and childhood they develop progressing kyphoscoliosis and developmental delay. Molecular analysis performed on DNA of our patient (and both parents) showed evidence of a new pathogenetic homozygous mutation never described before in the literature. Our case is one of 9 new mutations found in addition to 29 already described mutations, thus expanding the mutational spectrum of CRLF1 in Crisponi syndrome.

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Background: β-thalassemia is a genetic disorder of hemoglobin synthesis, which is not uncommon in world's population. According to international studies, platelet aggregation is increased in splenectomized patients while it is decreased or normal in non-splenectomized adult patients and children. These findings are one of the indications of hypercoagulable state in β-thalassemia patients, which leads to thromboembolic disease. Aims: To determine the frequency of platelet aggregation defects in children (aged 6 months to 5 years) suffering from β-thalassemia. Settings and Design: This study was conducted in Department of Hematology and Transfusion Medicine of the Children's Hospital and Institute of Child Health, Lahore. It was a cross-sectional study. Subjects and Methods: We selected 100 β-thalassemia patients of age between 0 and 5 years. History taking, examination, and investigations like complete blood count (CBC), Hb-electrophoresis on High Performance Liquid Chromatography (HPLC), serum iron, and platelet aggregation were performed on these patients. Results: During the study period, 100 children fulfilling the inclusion criteria were selected, and platelet aggregation with ADP, collagen, and ristocetin, was performed. It was found out that 66, 62, and 69% of the children showed decreased aggregation with ADP, collagen, and ristocetin, respectively, while 34, 38, and 31% children were found to have normal aggregation with ADP, collagen, and ristocetin, respectively. Conclusions: It was found that majority of the children showed decreased aggregation with ADP, collagen and ristocetin.

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Endometriosis is described as the presence of functioning endometrial tissue (glands and stroma) outside the uterine cavity. The most common location is within the pelvis. However, extrapelvic endometriosis is a fairly uncommon disorder, and is difficult to diagnose. It can sometimes occur in a surgical scar. Scar endometriosis is a rare condition. It mostly follows obstetrical and gynecological surgeries. It presents as a painful, slowly growing mass in or near a surgical scar. It is often misdiagnosed, leading to unnecessary referrals. Gynecologists and general surgeons must be aware of this entity to avoid discomfort to the patient due to delay in diagnosis.

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Background: Myxomas are the most common type of cardiac tumors in all age groups, and symptoms are produced by mechanical interference with cardiac function or embolization. This study comprises the clinical profile, surgical approach, morbidity, mortality and recurrence rate of atrial myxomas in the Kashmir valley. Patients and Methods: The study was retrospective from January 1991 to July 2005 and prospective from August 2005 to December 2007. All patients admitted with the diagnosis of atrial myxomas were included in the study. Results: The study included 30 patients with a mean age of 52 ± 4.8 years. Females outnumbered males, with a ratio of 2.9:1. Dyspnea was the most common symptom on presentation, with majority of the patients belonging to New York Heart Association Class III (56.6%). Pre-operative diagnosis was established in all patients by echocardiography. Majority of myxomas, i.e. 22 (88%), arose from interatrial septum. Wide excision of tumor with polytetrafluorethylene (PTFE)/pericardial patch closure was the most common surgical procedure performed. Both early and late mortalities were 6.6%. In addition to clinical examination, periodic echocardiography was done on follow-up to detect recurrence, which was seen in one (3.3%) patient. Conclusions: Surgical management is curative with minimal early and late morbidity and mortality. Extensive resection of the myxoma attached to the atrial septum or atrial wall can reduce the likelihood of recurrence to a great extent, and long-term clinical and echocardiographic follow-up is mandatory.

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Purpose: To evaluate the rate of myopic shift during 3.5 years in children with pseudophakia at a tertiary eye care hospital. Materials and Methods: A retrospective review of the medical records was conducted for children aged 1-9 years who underwent cataract extraction with intraocular lens (IOL) implantation in order to evaluate initial and annual postoperative refractions. Adjusted estimates of the rate of refractive shift related to patient characteristics were based on a generalized estimating equation model of spherical equivalent refractive error over time, taking into account time-related correlations within the data. Results: A total of 138 eyes of 117 subjects were included in the study. A statistically significant shift in refractive error over time was found in the myopic direction (P < 0.001) Significant interaction was present, indicating that age at time of surgery influenced the rate of myopic shift, but only in children with traumatic etiology. A myopic shift of -0.66 diopters/year was calculated in patients with cataracts of nontraumatic etiology. In those with traumatic cataracts, the rate of myopic shift was -0.85 diopters/year, with a decrease 0.13 diopters/year for each year of increasing age at time of surgery. Conclusion: Our findings suggest a trend toward myopia in pseudophakic children. This was particularly true in children with cataracts of traumatic origin. The strategy of aiming for a hyperopia in order to offset some of the expected myopia may be a reasonable one. Further work is required to develop the necessary nomograms.

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Background: Diffuse large B-cell lymphoma (DLBCL) is characterized by a marked degree of morphologic and clinical heterogeneity. BCL-6 gene rearrangements are the most common frequent chromosomal abnormalities in DLBCL. Genetic modifications of the BCL-6 gene in lymphoma include translocations, deletions, and somatic mutations (SM) of the 5′-non-coding region. Mutations in the 5′-regulatory region of BCL-6 were suggested to play a role in non-Hodgkin's lymphoma (NHL) progression. Materials and Methods: We studied single nucleotide polymorphism (SNP) at position 397(G->C) of the 5'-non-coding regulatory region of BCL-6. We examined 30 patients with DLBCL treated at the National Cancer Institute (NCI), Cairo University, and 21 normal lymph nodes from patients with breast cancer were selected as controls. We used (Polymerase chain reaction- Restriction fragment length polymorphism) PCR-RFLP coupled analysis to detect the polymorphism. Results: From 30 patients with DLBCL, 17 were males (56.7%) and 13 were females (43.3%), the polymorphism G397C in the 5′ region of BCL-6 was detected in seven cases (23.3%). There was no significant association of clinical characteristics of DLBCL patients with this polymorphism, but there a trend for this polymorphism to occur more frequent in patients with aggressive disease. Conclusion: In our study, we found the incidence of the G397C SNP mutations in the 5′-regulatory region of BCL-6 to be 23.3%. No statistically significant association between the G397C SNP in BCL-6 gene with the standard clinico-pathological factors in patients with DLBCL was observed; however, there a trend for this polymorphism to occur more frequent in patients with aggressive disease.

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Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome which is mostly associated with pre-eclampsia, autoimmune diseases, and cytotoxic medication. Our understanding of this disease is based mainly on case reports which identify causes, pattern, and prognosis, and the reports in children are not frequent. We report a case of acute glomerulonepheritis and PRES, which presented with generalized convulsions and loss of consciousness. On diffusion-weighted magnetic resonance imaging (MRI), the affected areas appeared hyperintense on both diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps, which is consistent with unrestricted diffusion in regions of PRES vasogenic edema. MR spectroscopy (MRS) revealed normal metabolite spectrum. Our case was completely reversible after blood pressure control. We conclude that PRES as a possibility should be considered in children with acute glomerulonepheritis with neurological findings, even with status epilepticus presentation, and that we can confirm our suspicion by MRI showing typical bilateral hypodense findings.

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Background: Coronary atherosclerosis is a disease of the coronary arteries and is a result of cumulative processes that are mostly seen in old-age population. Progressive accumulation of oxidative damage to cellular macromolecules, especially DNA, over a lifetime is one of the important causes of aging. This accumulation of damage may result in chromosome instability and this can be measured by micronucleus (MN) technique. Materials and Methods: In this study, MN technique was applied to observe the relationship between chromosome instability and proliferative effectiveness of lymphocytes and coronary atherosclerosis. Non-toxic levels of hydrogen peroxide were also administered to observe any existing differential effect due to this stress inducing oxidizing agent. Spontaneous and H ₂ O ₂ stress induced MN frequencies and proliferation state of peripheral lymphocytes were measured from both 30 patients diagnosed as coronary atherosclerosis and 30 healthy control individuals of the same age range. Results: There was no significant difference between spontaneous MN frequencies of patient and control groups (P = 0.08). Also, probability index (PI) values between these groups reflected no difference (P = 0.13). Hydrogen peroxide addition did not change spontaneous MN frequencies either in control (P = 0.23) or in patient group (P = 0.81). This non-toxic H ₂ O ₂ level failed to show any different effect on patients compared to controls (P = 0.41). Although increasing PI values were observed as a result of H ₂ O ₂ administration only in patient group, the comparisons between the two groups showed no significant difference (P = 0.34). Conclusion: Chromosome instability and proliferation index that were measured by MN technique in this study have no role in discriminating coronary atherosclerosis patients from healthy individuals.

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Objective: Thousands of millions of injections are unsafely administered and disposed every year in developing countries, which may pose hazardous consequences to the environment as well as public. The prime objective of this study was to determine the perception and attitudes of the Sudanese patients about safe use and disposal of injections. Materials and Methods: A cross-sectional study was carried out. One thousand patients were randomly selected from different health settings in Sudan. Data collection was carried out during the period from September 2009 to March 2010. Results: The majority of the respondents (95.1%) admitted that they had received medicines in injection form through their lives. Nurses were the dominant injection administrators (42.5%), while the doctors formed 19.8%. Majority of the interviewed patients had their injections at homes (38.7%). In the present study, 72.9% of the patients used to take drugs in injection by using sterile disposable syringes. Unfortunately, around 90% of the used injections found their way to inappropriate disposal places. These unaccepted injection disposal practices led to a considerable rate of used injection sticks (26.1%). Conclusions: A set of recommendations was proposed in an attempt to educate patients on the safe use and disposal of injections.

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