Cases of neuro-ferritinopathy from Sudan

Neuro-ferritnopathy (NBIA) is a bag of diseases due to abnormal iron metabolism. It has different underlying genetic and enzymatic abnormalities. On the other hand, they share some radiological features. Patients present with a wide range of cerebral symptoms and signs. Diagnosis depends on the semiology, genetic testing and MRI imaging. No specific treatment is available for these cases and they represent a challenge to the treating neurologist. Here we illustrate two interesting cases with their clinical and imaging findings to raise the awareness of such rare diseases and help diagnosing them in a low- resource setting

Pneumosinus Dilatans – A Rare Disorder Of Paranasal Sinuses

Pneumosinus dilatans is a very rare disorder of the paranasal sinuses, most commonly affecting the frontalsinuses (Pneumosinus dilatans frontalis). So far only 134 cases are reported in the literature. The exact etiologyof this disorder is unknown. The pathology causes abnormal dilatation of paranasal sinuses. Most patients areasymptomatic while others can have clinical features of frontal bossing, head ache, visual defects etc. Thediagnosis is by computed tomographic imaging. We here report a case of pneumosinus dilatans frontalis in a 22year old male patient presented with nasal obstruction.

Inhibition of retroviral Gag assembly by non-silencing miRNAs promotes autophagic viral degradation

We recently reported an unconventional mechanism by which miRNAs inhibit HIV-1 viral production. This occurs when miRNAs bind nonspecifically to the viral structural protein Gag, interfering with viral RNA-mediated Gag assembly at the plasma membrane. Consequently, misassembled viral complexes are redirected into the endocytic pathway where they are delivered to lysosomes for degradation. In this study, we demonstrate that autophagy is a critical mediator of the viral degradation pathway and that this pathway is not HIV-1 specific. Misassembled viral complexes were found to colocalize extensively with LC3 and p62 in late endosomes/lysosomes, demonstrating a convergence of autophagy with functional degradative compartments. Knocking down autophagosome formation machineries reduced this convergence, while treatment with autophagy-inducer rapamycin enhanced the convergence. Furthermore, similar autophagy-dependent nonspecific miRNA inhibition of murine leukemia virus (MLV) assembly was shown. Overall, these results reveal autophagy as a crucial regulator of the retroviral degradation pathway in host cells initiated by nonspecific miRNA-Gag interactions. These findings could have significant implications for understanding how cells may regulate retroviral complex assembly by miRNA expression and autophagy, and raise the possibility that similar regulations can occur in other biological contexts.

Risk factors, post-operative complications and outcome of management of hip fracture in arar, northern saudi arabia

Background: Hip fractures are defined as any fracture of the femur between the articular cartilages of the hip joint to 5 cm below the distal point of the lesser trochanter. Hip fracture is a worldwide public health problem that primarily affects osteoporotic individuals and the elderly. Up to 30 % of the elderly patients with a hip fracture die within the first year

Objective: to show the risk factors and post-operative complications of hip fracture in cases attending orthopedic department of Arar Central Hospital in Arar city

Methods: a cross sectional study conducted during the period from 1 December 2017 to 31 March 2018. A predesigned questionnaire was used for data collection, and included inquiries about sociodemographic data of the studied patients, performing muscular exercise, osteoporosis, diminished vision and disorders in equilibrium, causes of fracture, type and site of fracture, occurrence of complications of surgery, postoperative care and the final outcome of treatment

Results: Most [70.1%] of the studied population aged 22-59 years, males constituted 59.8%. Causes of hip fracture were accident in 66.7% and fall in 25.0%. Among hip fracture cases, 43.9% were obese, 13.1% have osteoporosis, and 17.8% have disorders in equilibrium. Males reported insignificant higher percentage of hip fracture than females [25.0% Vs. 18.6%]. Osteoporosis, diminished vision, chronic diseases, continuous use of medications and smoking showed significant relation with hip fracture [P<0.05]. While BMI, disorders in equilibrium and performing muscular exercise showed insignificant relation. Complications after surgery was osteomyelitis in 12.6%, early fixation failure in 4.2%, wound infections in 8.4% and hospital acquires pneumonia in 4.2%. Only two thirds of the cases were completely cured

Conclusion: in our study population in Arar city, males reported insignificant higher percentage of hip fracture than females. Osteoporosis, diminished vision, chronic illnesses, continuous use of medications and smoking were significant risk factors of hip fracture. Complications after surgery was included, osteomyelitis, early fixation failure, wound infections and hospital acquire pneumonia. Only two thirds of the cases were completely cured, the rest of cases showed disability and movement limitation

Congenital hydrocephalus in Arar, Northern Saudi Arabia

Background: Congenital hydrocephalus, an important cause of neurologic morbidity and mortality in children, is a medical condition characterized by an abnormal accumulation of cerebrospinal fluid in the brain

Objectives: To estimate the prevalence and the recent progresses in diagnosis of hydrocephalus as well as the changes in epidemiology and treatment outcomes of the disease

Methods: This is a descriptive study involved all neonates born in Maternity and child hospital in Arar city, Northern Saudi Arabia, KSA, during the period from 1 January to 31 December 2017. Among 6000 delivered infant in 2017; there was 23 cases of hydrocephalus. Data was collected by using predesigned questionnaire which include questions designed to fulfill the study objectives

Results: the prevalence of hydrocephalus among studied infants was 0.38%. Consanguinity between parents was reported among 60.9% of the cases. Other congenital anomalies reported, cardiac anomalies 39.1%, spina bifida 17.4% and cleft palate 8.7%. Cause of hydrocephalus was 65.2% hereditary and 34.8% infection [secondary cause]. Swelling of the eyelid, increased vascular clarity on the skull, transparent skin in the head, the child's tendency to sleep, disorientation, irritability and nervousness, high crying, weakness of feeding and vomiting, delayed child skills, convulsions, increased size of the head, the sun's sign in the eye and the child is always looking down were the symptoms reported by the cases. 56.5% of the cases had shunt insertion and 43.4% had ordinary medical treatment. 30.4% of the shunt cases were complicated [17.4% had shunt blockage with infection and fever, 13% shunt blockage and 69.6% had other complications]. Outcome of the case; 39.1% were stable, 13% worsen and 47.8% died [34.7% died by complications, 8.7% during operation and 4.3% did not receive treatment]

Conclusion: The prevalence of hydrocephalus is comparable to that of other countries. More research is recommended because it is probable that the real prevalence of congenital hydrocephalus was underestimated and no account of how often abortions are performed among mothers of hydrocephalic fetuses. So more efforts from the ministry of health is needed

Role of Transesophageal Echocardiography in the Diagnosis of Paradoxical Low Flow, Low Gradient Severe Aortic Stenosis

BACKGROUND AND OBJECTIVES: Prior studies indicate that up to 35% of cases of severe aortic stenosis (AS) have paradoxical low flow, low gradient despite preserved left ventricular ejection fraction (LVEF). However, error in left ventricular outflow tract (LVOT) diameter may lead to misclassification. Herein, we determined whether measurement of LVOT diameter by transesophageal echocardiography (TEE) results in reclassification of cases to non-severe AS. SUBJECTS AND METHODS: Patients with severe AS with aortic valve area (AVA) <1 cm2 by transthoracic echocardiography (TTE) within 6 months were studied. Paradoxical low flow, low gradient was defined as mean Doppler gradient (MG) <40 mm Hg and stroke volume index (SVI) ≤35 mL/m². Preserved LVEF was defined as ≥0.50. RESULTS: Among 108 patients, 12 (15%) had paradoxical low flow, low gradient severe AS despite preserved LVEF based on TTE measurement. When LVOT diameter by TEE in 2D was used, only 5 (6.3%) patients had low flow, low gradient severe AS (p<0.001). Coefficients of variability for intraobserver and interobserver measurement of LVOT were <10%. However, the limits of agreement between TTE and TEE measurement of LVOT ranged from 0.43 cm (95% confidence interval [CI]: 0.36 to 0.5) to -0.31 cm (95% CI: -0.38 to -0.23). CONCLUSION: TEE measured LVOT diameter may result in reclassification to moderate AS in some patients due to low prevalence of true paradoxical low flow, low gradient (PLFLG) severe AS.

Anomalous coronary artery in a transplanted heart: a rare incidental diagnosis

Coronary artery anomaly is a rare postoperative coronary angiographic finding in heart transplant recipients. We report a case of anomalous origin of the right coronary artery in an asymptomatic 70-year-old heart transplant patient. Most coronary artery anomalies are benign, but surgical treatment may be necessary in major coronary artery anomalies that are known to have adverse outcomes

Paradoxical coronary artery embolism - a rare cause of myocardial infarction

Paradoxical coronary artery embolism is a rare, but often an underdiagnosed cause of acute myocardial infarction. It should be considered in patient who presents with chest pain and otherwise having a low risk profile for atherosclerosis coronary artery disease. We describe a case of paradoxical coronary artery embolism causing ST segment elevation myocardial infarction in a patient with upper extremity venous thrombosis. Echocardiography demonstrated a patent foramen ovale [PFO] with bidirectional shunt. In addition to treatment of acute coronary event closure of the PFO should be considered to prevent a recurrence

A study of in vitro antibacterial activity of lanthanides complexes with a tetradentate Schiff base ligand

<p><b>OBJECTIVE</b>To establish the antibacterial activity of lanthanides complexes with a tetradentate Schiff base ligand L.</p><p><b>METHODS</b>(N, N'-bis (1-naphthaldimine)-o-phenylenediamine) was prepared from the condensation of 2-hydroxy-1-naphthaldehyde with o-phenylenediamine in a molar ratio of 2:1. The antimicrobial activity of the resultant Ln (III) complexes was investigated using agar well diffusion and micro-broth dilution techniques; the latter was used to establish the minimum inhibitory concentrations for each compound investigated.</p><p><b>RESULTS</b>Most of Ln (III) complexes were found to exhibit antibacterial activities against a number of pathogenic bacteria with MICs ranging between 1.95-250.00 µg/mL. Staphylococcus aureus was the most susceptible bacterial species to [LaL(NO3)2(H2O)](NO3) complex while Shigella dysenteriae and Escherichia coli required a relatively higher MIC (250 µg/mL). The complexes La (III) and Pr (III) were effective inhibitors against Staphylococcus aureus, whereas Sm (III) complex was effective against Serratia marcescens. On the other hand, Gd (III), La (III) and Nd (III) were found to be more potent inhibitors against Pseudomonas aeruginosa than two of commonly used antibiotics. The remaining Ln (III) complexes showed no remarkable activity as compared to the two standard drugs used.</p><p><b>CONCLUSIONS</b>Tetradentate Schiff base ligand L and its complexes could be a potential antibacterial compounds after further investigation.</p>

A study of in vitro antibacterial activity of lanthanides complexes with a tetradentate Schiff base ligand

Objective: To establish the antibacterial activity of lanthanides complexes with a tetradentate Schiff base ligand L. Methods: (N, N'-bis (1-naphthaldimine)-o-phenylenediamine) was prepared from the condensation of 2-hydroxy-1-naphthaldehyde with o-phenylenediamine in a molar ratio of 2:1. The antimicrobial activity of the resultant Ln (III) complexes was investigated using agar well diffusion and micro-broth dilution techniques; the latter was used to establish the minimum inhibitory concentrations for each compound investigated. Results: Most of Ln (III) complexes were found to exhibit antibacterial activities against a number of pathogenic bacteria with MICs ranging between 1.95-250.00 μg/mL. Staphylococcus aureus was the most susceptible bacterial species to [LaL(NO3)2(H2O)](NO3) complex while Shigella dysenteriae andEscherichia coli required a relatively higher MIC (250 μg/mL). The complexes La (III) and Pr (III) were effective inhibitors against Staphylococcus aureus, whereas Sm (III) complex was effective against Serratia marcescens. On the other hand, Gd (III), La (III) and Nd (III) were found to be more potent inhibitors against Pseudomonas aeruginosa than two of commonly used antibiotics. The remaining Ln (III) complexes showed no remarkable activity as compared to the two standard drugs used. Conclusions: Tetradentate Schiff base ligand L and its complexes could be a potential antibacterial compounds after further investigation.

Splenic lymphoma in HCV positive patient; a case report

Splenic Lymphoma is a marginal zone lymphoma [MZL's] that is a type of Non-Hodgkirf s Lymphoma. It is characterized by a lymphocytic tumor of marginal zone of spleen that may involve splenic hilar lymph nodes and may spill the abnormal B-Lymphocytes in the blood. A 3 8 years old male, known HCV, presented with pain in left hypochondrium and an abdominal mass in left hypochondriac region

He was diagnosed clinically as a case of splenic hematoma which turned to be splenic lymphoma after investigations and was treated

Preliminary report on the Neurology workload ina central hospital in Sudan

Identification of the local pattern of neurological diseases is expected to help setting the priorities for good planning of management and public education. To identify the pattern of neurological disorders in a second biggest central hospital in Sudan. Review of the diagnosis of 170 patients, seen in the out patients neuro-clinic and the neurology ward from March 2010 to February 2011, was done. Patients who were in follow up by other neuro-clinics were excluded. The male to female ratio was 1:1 Patients who were 20-60 years old comprised 54%.About 50% of patients live in Omdurman city. Motor symptoms were the most common presenting symptoms forming 64.1% followed by cranial nerves symptoms 27.6%. Stroke was the commonest encountered diagnosis seen in 20.7%, followed by epilepsy in 16.6%, headache in 9.6%, movement disorders in 7.7%, peripheral neuropathy in 3.6%, demylination in 1.8%, cerebral venous sinus thrombosis [CVST] 1.8% and tumours in 1.2% patients. Stroke was more common in patients from East Sudan and Epilepsy was more common in patients from West Sudan. Vascular and degenerative changes were the commonest abnormalities seen in imaging studies. Demyelination and neoplasms were more common in females. Stroke, epilepsy and headache were the commonest neurological disorders met in Omdurman Teaching Hospital

Patterns of pituitary adenoma among adult Sudanese patients

The objective of this study is to determine the clinical presentation and hormonal secretions of patients with pituitary adenomas. This is a descriptive, prospective study on 53 adult Sudanese patients with pituitary adenoma. Only adults [16 years or more] were included. The study was conducted in Al-Shaab Teaching Hospital in the period from January 2001 to February 2007. Twenty eight [52.8%] patients were females and 25 [47.2%] were males. Their ages ranged between 16 -80 years [mean 40.8 +/- 15.6 years]. Twenty seven [50.9%] patients had functioning pituitary adenomas [FPA] and 26 [49.1%] patients had nonfunctioning pituitary adenomas [NFPA]. Hormonal secretion of the adenomas was found to be prolactin in 16 patients [14 females and 2 males], growth hormone in 8 patients [3 females and 5 males], ACTH in 2 patients [one female and one male] and TSH in one female. Macroadenoma was found in 44 patients [83%], while microadenoma was found in 9 patients. All non functioning adenomas were found to be macroadenomas, while functioning adenomas were found in 18 patients with macroadenoma and in 9 patients with microadenoma. In our series all NFPAS were macroadenomas. They were usually discovered late after assuming mass effect or incidentally on imaging for other reasons. Patients with functioning adenomas tend to present earlier with hormonal dysfunction and hence are diagnosed earlier before the tumors assume big sizes. Prolactin secreting tumors are the commonest among functioning pituitary adenomas and tend to predominate in females

Intracranial meningioma: findings from Sudan

The objective of this article is to determine the pattern of neurological presentation of meningiomain adult Sudanese patients. In this study we described the clinical presentation and neuroimaging findings in fifty patients with Intracranial meningioma. In all patients the diagnosis was histologically verified. The age distribution of the patients ranged between 19 -70 years, the mean age was 44.5 +/- 13.33 years. Females were 36 [72%] and males 14 [28%]. Male: female ratio was 1:2.6. The presenting symptoms were headache and nausea in 37 [74%] patients, behavioral changes in 34 [68%], visual disturbance in 23 [46%], generalized seizure in 20 [40%], limb weakness in 20 [40%], vomiting in 13 [26%], speech disturbance in 13 [26%], sphincteric disturbances in 11 [22%], focal seizures in 10 [20%], anosmia in 4 [8%], and deafness in 2 [4%]. Meningiomas were located in anterior fossa in 18 patients [40%], parasagittal in 9 [20%], sphenoidal ridge in 8 [18%], posterior fossa in 7 [15.9%], lateral convexity in 5 [11.3%], olfactory groove in 4 [9%], middle fossa in 2 [4.5%] and supraseller area in one [2.2%]. All meningiomas enhanced with gadolinium. Peritumoural oedema was found in 24 [54.5%] patients, hydrocephalus in 11 [25%] and hyperostosis in 9 [20.5%] patients. It has been concluded that the clinical presentation and MRI findings did not differ much from what is reported worldwide

Ataxia telangiectasia: findings from two families in the Sudan

We report herein two families with ataxia telangiectasia. Patients in both families fulfilled the diagnostic criteria and showed unusual presentations that are rarely reported in literature. No patient developed malignancy yet. To the best of our knowledge this is the first report on Ataxia telangiectasia from Sudan

Invasive aspergillosis of paranasal sinuses: findings from the Sudan

The Sudan is endemic for Aspergillus species, especially Aspergillus flavus. They commonly invade the paranasal sinuses, but massive invasion of the brain in immunocompetent patients is rare. To describe the clinical presentation and MRI findings in immunocompetent patients with massive paranasal aspergillosis with extensive invasion of the brain. Four patients, who are negative for HIV and other immunocompromizing disorders were studied in this article. Two females 55 and 25 year old, 2 males 29 and 62 year old were described. All cases showed extensive invasion of the paranasal sinuses and the adjacent structures. Invasive paranasal Aspergillosis can affect immunocompetent patients and the commonest organism is Aspergillus flavus

Pott's disease of the spine: findings from Sudan

Spinal tuberculosis is the commonest type of skeletal tuberculosis. It has variable presentations, but commonly presents with backache and weakness of lower limbs. Its prevalence is increasing world wide after the epidemic of HIV. To the best of our knowledge there are no studies addressing the clinical presentation of this disease in Sudan. In this study we included 84 patients with spinal tuberculosis. The study was conducted in two major hospitals in Khartoum in the period from January 2002 to December 2006. Results show males constituted 46 patients [54.8%] and females 38 [45.2%]. Their ages ranged between 16 years and 80 years, the mean age was 44.46 +/- SD 16.99. All patients had pain and or tenderness at the site of the lesion. Paraplegia or paraparesis was reported in 36 patients [42.8%], quadriplegia or quadriparesis in 25 [30%], Sciatica and root weakness in 22 [26.1%] and right brachial monoplegia in one patient. Evidence of active pulmonary tuberculosis was found in 15 patients [17.8%], abdominal tuberculosis in 7 patients [8.3%], lymphatic Tuberculosis in 1 patient [1.2%]. Cervical vertebrae were affected in a total of 26 patients [30.9%], the dorsal in 39 patients [46.4%] and the lumbar in 30 patients [35.7%]. One vertebra was affected in 3 patients [2.57%], 2 adjacent vertebrae in 48 patients [57.14%], 3 vertebrae in 24 patients [28.57%], 4 vertebrae in 4 patients [4.76%], 5 vertebrae in 1 patient [1.19%] and 6 vertebrae in 4 patients [4.76%]. HIV test was negative in all patients. Tuberculin test was positive in 80 patients [95%] and negative in 4 patients [5%]

Osteopetrosis: a rare disease with rare presentations

At least eight types of osteopetrosis have been described in humans. The primary underlying mechanism involved in all forms is the failure of normal osteoclastic bone resorption. Osteopetrosis tarda, the benign adult form, is inherited as an autosomal dominant trait. Patients typically are asymptomatic and have good long-term survival rates because bone marrow failure rarely occurs. A more common and malignant form, osteopetrosis congenita, presents in infancy and results in bone marrow failure caused by complete replacement of the marrow spaces with osteoclasts. Other forms are rare and have variable manifestations. In this communication we report 2 cases on possibly a rare form of osteopetrosis, the intermediate form