Rhinocerebral Mucormycosis Following COVID-19 Infection in Iran.

Background & Objective: Mucormycosis (also called black fungus) is an opportunistic serious fungal infection caused by mucormycetes. It can occur in diabetes mellitus patients and other immunosuppressive conditions with recent predisposing factors such as maxillofacial surgery and corticosteroid usage. Methods: In this study, 14 patients were referred to the otorhinolaryngology or ophthalmology ward of Shafa Hospital (Kerman, Iran) with primary symptoms of nasal fullness and facial nerve dysfunction; they were admitted to the hospital to rule out the fungal infection. An endoscopic biopsy was taken from facial sinuses or orbit, and a microscopic evaluation was performed using hematoxylin and eosin (H&E) and periodic acid-Schiff (PAS) staining methods to rule out mucormycosis. Results: In the histopathological examination, broad-based nonseptate branching fungal hyphae were found in nasal sinuses through the endoscopic biopsy. Most of the patients had diabetes mellitus with a primary symptom of facial nerve palsy; also, most of them received corticosteroids (intravenous [IV] or intramuscular [IM] injection). All patients have recently been infected with COVID-19 (less than 1 month ago). Conclusion: COVID-19 infection might be a predisposing factor for many opportunistic infections, such as fungal elements); thus, the physician should be aware of the dosage and duration of corticosteroid therapy to prevent the development of these infections.

Supplementary suckling technique in infants less than 6 months of age with uncomplicated severe acute malnutrition: a prospective hospital-based study in armed conflict Yemen.

BACKGROUND: Globally 3.8 million of children under 6 month of age are severely wasted. In Yemen, around 20% of children under 6 months were affected by malnutrition during the armed conflict in the last 7 years. Supplementary suckling may reestablish exclusive breastfeeding in infant less than 6 months of age with Severe Acute Malnutrition (SAM). This study aimed to determine the outcomes of employing supplementary suckling technique in treatment of uncomplicated SAM infants in a conflict-affected community. METHODS: A prospective hospital-based study was carried out between January to April 19th, 2020 among randomly selected infants less than 6 months of age with SAM following breastfeeding failure. Infants' anthropometric indices were daily measured and recorded. Supplementary sulking technique was used in management with high or low protein milk-based formula supplement. Outcome was recorded as cured, died, defaulter or in nonrecovery state. RESULTS: In this study 108 infants were enrolled with a median (IQR) age of 4 (2.5-5) years and a male: female ratio of 1.4:1. After treatment, 80.6% recovered to cure, 12% defaulters, 6% died, and 2% did not respond to treatment Thirty-four infants (38.8%) gained weight with significantly increased median weight and median weight-for-age z score. The median (IQR) duration of treatment was 9 (7.5-14) days. The means of age and weight-for-length z score were correlated (r = - 0.22, p = 0.025). Duration of treatment was a predictor of outcome (OR = 1.71, 95% CI = 0.05-0.62, p < 0.001). CONCLUSION: Supplementary suckling technique for feeding infant with SAM aged less than 6 months had a positive impact on anthropometric indices with high cure rate. The younger the infant and the longer the duration of treatment, the better the outcome.

The Role of Optical Coherence Tomography in Early Detection of Retinal Nerve Fiber Layer Damage in Pituitary Adenoma.

Background and objectives:This study aimed to evaluate the ability of optical coherence tomography (OCT) to differentiate eyes without obvious visual disturbances following pituitary adenomas (PAs) from normal eyes, in order to identify factors that could predict early diagnosis and timely treatment and prevent structural damage of visual pathway in patients with saddle area tumors. Material and methods:The present study was carried out between 2014-2018. Participants were divided into three groups: 23 subjects (44 eyes) in the PAs with visual field involvement (VFI) group, 10 (20 eyes) in the PAs with normal visual field (NVF) group and 22 subjects (44 eyes) in the control group. All patients received diagnostic magnetic resonance imaging (MRI), automated perimetry, visual acuity, OCT and ophthalmological assessments. Also, the degree of visual field (VF) deficit and thickness of peripapillary retinal nerve fiber layer (pRNFL) were measured by OCT and then considered for statistical analysis as predictors of early diagnostic visual involvement in the PAs. Results:All patients in the NVF and control groups (a total of 64 eyes) had normal VF. In the VFI group there were 16 eyes with complete hemianopia. Bitemporal hemianopia occurred in 20 eyes and eight eyes with concentric VF narrowing. Assessment of pRNFL thickness with OCT demonstrated the average and all quadrants of pRNFL thickness in the VFI group were significantly thinner than the pRNFL thickness in the other groups (P<0.001). The pRNFL thickness in the inferior and nasal quadrants and the average value in the NVF group were significantly thinner than the control group (P<0.05). Conclusion:If a patient has band atrophy, there is an irreversible damage and the main goal is to diagnose a nerve damage using OCT before band atrophy.

Evaluation of dietary compliance among Sudanese children with coeliac disease.

The compliance to a gluten-free diet in Sudan is difficult because many Sudanese tribes depend on wheat product for their food with no access to gluten-free food. To evaluate dietary compliance among Sudanese children with coeliac disease (CD), a longitudinal, prospective and hospital-based study was carried out at Ibn Sina Hospital (Coeliac Clinic) and Gaafar Ibn Auf Children's Hospital (Gastroenterology Clinic) from July 2011 to February 2012. Fifty children diagnosed with CD, followed for more than 6 months, were assessed for dietary compliance. Dietary good-compliant and poor-compliant groups were compared to assess factors affecting the dietary compliance. History, clinical examination and investigations were done initially and then 6 months later. Diagnosis of CD was based on serological tests and duodenal biopsy. The mean age of children with CD was 6.5 (± 2) years. More than three quarters of participants (38%-76%) had poor dietary compliance. Causes of poor compliance were as follows: in 7 (18.4%) was due to refusal of children, in 9 (23.7%) due to unpalatability of gluten free diet (GFD), in 12 (31.6%) due to difficulty in finding GFD and in 10 (26.3%) due to lack of awareness of parents about CD and GFD. The study showed significant correlation between the initial and after 6-month follow up, and the following measures: clinical assessment, nutrition status and difference in haemoglobin level (p < 0.05). To improve compliance, a team working among families is needed with specialised dietitian as well as follow-up at specialised CD clinics.

Fanconi Bickel syndrome: clinical phenotypes and genetics in a cohort of Sudanese children.

BACKGROUND: Fanconi-Bickel syndrome (FBS) is a rare condition of carbohydrate metabolism, caused by a recessive defect in the facilitative glucose transporter GLUT2 encoded by the SLC2A2 gene and characterized by a wide spectrum of phenotypical features. There is a paucity of reported data on FBS from Sub-Saharan Africa. Here, we describe the clinical, biochemical and genetic characteristics of our patients with FBS from Sudan, a country with a high consanguinity rate. PATIENTS & METHODS: Eleven patients from ten unrelated Sudanese families were included. Clinical & biochemical data were documented and imaging studies done including bone survey and abdominal ultrasound. Liver biopsy was done to confirm the pathological diagnosis in 45% of cases and molecular genetics was performed through contribution with the Exeter genomics laboratory for ten patients. RESULTS: Reported consanguinity was 70% among our patients. Growth was significantly impaired at presentation with mean weights of (-5.3 ± 1.8) SD and heights (-5.4 ± 2.5) SD. Severe chest deformity was present in (27%) and all patients showed features of rickets at presentation. Three patients had neonatal diabetes requiring insulin therapy of which one has been reported before. Six families lost undiagnosed siblings with similar clinical presentations. We identified a total of four homozygous pathogenic SLC2A2 variants in our patients, one of whom had a novel mutation. CONCLUSIONS: FBS is not uncommon in Sudan where there is a high rate of consanguinity. Many cases are likely missed because of variable presentation and lack of public and professionals' awareness. This is the first series to describe this condition from Sub-Saharan Africa.

Maternal depression, a hidden predictor for severe acute malnutrition in children aged 6-59 months: a case-control study at Omdurman Paediatrics Teaching Hospital, Sudan.

Malnutrition remains one of the main disabling issues in child health, especially in developing countries. Maternal depression by its related disabilities has been linked with children undernutrition in the studies abroad. Unfortunately, not much is known regarding this issue in Sudan, so this study aims to examine the association between maternal depression and severe acute malnutrition (SAM) in children under 5 years of age. A matched case-control study was conducted in Omdurman Paediatrics Teaching Hospital. Children admitted with SAM were assigned as cases, whereas controls were age- and sex-matched children with normal weight and height admitted in the same hospital. Mothers of both cases and controls were assessed for depression utilising the Patient Health Questionnaire-9 tool. The prevalence of depression among mothers of malnourished children was high (41.5%) compared to the mothers of controls (19.1%). In multivariate logistic regression analyses, the adjusted odds ratio (AOR) of maternal depression were markedly higher in cases than in controls (AOR = 3.09, p = 0.002), as was the odds of below 1-year breastfeeding weaning (AOR = 18.60, p = 0.006) and mother illiteracy (AOR = 2.42 p = 0.031). Furthermore, the analysis found a significant negative association between the occurrence of malnutrition and exclusive breastfeeding (AOR = 0.43, p = 0.015). Maternal depression carries a significant burden in the mothers of children hospitalised with SAM. We strongly recommend routine screening and treatment for depression in childbearing age mothers in the available relative maternal and child health clinics.

Clinical presentation of coeliac disease and the effect of sorghum-based diet on anthropometric measurements among Sudanese children.

Coeliac disease (CD) is a chronic enteropathy. Sorghum (Sorghum vulgaris) is a common staple in Sudan. The literature on the growth of children with CD following sorghum diet is scanty. The aim of this study was to identify the demographic and clinical characteristics of CD in children and to determine the anthropometric response to a diet based on sorghum. This prospective study included children with probable CD in Ibn Sina Hospital, Khartoum, Sudan, from January 2002 to February 2012. The demographic, anthropometric, clinical and laboratory data were recorded. The CD serology was done, and the diagnosis of CD was based on Marsh criteria. Children who consumed sorghum and their anthropometry were recorded at 3 and 6 months after diagnosis. Children enrolled were 218. The mean ± SD age was 8.2 ± 4.5, median was 7 years and female/male ratio was 1.12/1. CD was common among Nubians and Arabs. The majority (145, 66.5%) presented with gastrointestinal symptoms. Feeding on sorghum diet resulted in a significant increase in weight after 3 and 6 months (21.1 ± 9.8 and 25.1 ± 14.2 kg, respectively) of the initial visit (18.5 ± 9.4 kg), p < 0.001 and p = 0.001, respectively. The mean weight for height had significantly increased at the second compared to the initial visit (0.17 ± 0.05 vs. 0.15 ± 0.5), p < 0.001. There was no association between gaining weight and age, gender, or a family history of CD. In conclusion, Sudanese children with CD presented over 8 years of age. The common presentation was gastrointestinal symptoms. The initial weight and weight-for-height increment were significant on sorghum diet.

Perinatal outcomes following Helping Babies Breathe training and regular peer-peer skills practice among village midwives in Sudan.

BACKGROUND: Over 80% of deliveries in Sudan occur in rural areas, attended by village midwives (VMWs). OBJECTIVE: To determine the impact of Helping Babies Breathe training and regular peer-peer skills practice (HBBT+RPPSP) on VMW resuscitation practices and outcomes. METHODS: In a prospective community-based intervention study, 71/82 VMWs, reporting to six East Nile rural medical centres, with previous experience in community health research, consented to HBBT+RPPSP. Outcomes included changes in the resuscitation practices, fresh stillbirths (FSB) and early neonatal deaths <1 week (ENND). RESULTS: There were 1350 and 3040 deliveries before and after HBBT+RPPSP, respectively, with no significant differences between the two cohorts regarding maternal age, education or area of birth. Drying of the newborn increased almost tenfold (8.4%, n=113 to 74.9%, n=1011) while suctioning of the mouth/nose decreased fivefold (80.3%, n=2442 to 14.4%, n=437) following HBBT+RPPSP. Pre-HBBT+RPPSP9/18 (50%) newborns who had mouth-to-mouth ventilation died, compared with 13/119 (11%) who received bag-mask ventilation post-HBBT+RPPSP. Excluding 11 macerated fetuses, there were 55 perinatal deaths: 14 FSB/18 ENND (6 months pre-HBBT+RPPSP) and 10 FSB/13 ENND (18 months post-HBBT+RPPSP). FSB rates decreased from 10.5 to 3.3 per 1000 births ((χ2)=8.6209, p=0.003), while ENND rates decreased from 13.5 to 4.3 per 1000 live births ((χ2)=10.9369, p=0.001) pre-HBBT+RPPSP and post-HBBT+RPPSP, respectively. CONCLUSION: In a selected group of VMWs, HBBT+RPPSP was associated with improvements in newborn resuscitation and perinatal outcomes. HBBT+RPPSP could have immense benefits if propagated nationally to all 17 000 VMWs in Sudan.

Skills retention in Sudanese village midwives 1†year following Helping Babies Breathe training.

BACKGROUND: Over 80% of deliveries in Sudan occur in isolated villages, attended by village midwives (VMWs). Upgrading newborn resuscitation skills with the Helping Babies Breathe (HBB) programme could improve newborn survival rates. OBJECTIVE: To describe the competencies in newborn resuscitation of selected VMWs pre-HBB and post-HBB training. METHODS: In a prospective intervention study, the VMWs' performances in the HBB Objective Structured Clinical Examination B simulated scenario (manikin requiring face-mask ventilation (FMV)) were digitally recorded and analysed prior to and 3 and 12 months following HBB training. Regular manikin-based practice was encouraged following training. RESULTS: Pre-HBB training, 42% of 71 VMWs (of whom 61% were functionally illiterate) stimulated the non-breathing manikin by holding it by the legs and either stimulated/slapped (30.4%) or shook (12.7%) it, while 25% (18/71) provided manikin mouth-to-mouth ventilation. The low scorings on the 'preparation for birth' (0% and 3.1% at 3 and 12 months, respectively) were mainly due to failure to demonstrate the subitem of 'cleans hands'. The percentage of VMWs providing manikin FMV within the Golden Minute increased from 37.3% (25/67) to 72.3% (47/65) (p<0.005), but there were no significant differences in the number of VMWs producing at least five FMVs at 3 months (73%, 49/67) and 12 months (58%, 38/65), respectively. CONCLUSIONS: VMWs, despite a high illiteracy rate, absorbed and sustained HBB skills for at least a year. Regular, low intensity, manikin-based skills training with peers may have helped sustain FMV, but not hand-cleansing skills.

Cystic fibrosis in Sudanese children: First report of 35 cases.

Cystic fibrosis is the most common severe genetic disorder among children of European descent. It is much less common in Africans and Asians. It affects most critically the lungs causing chronic lung disease, failure to thrive and social deprivation. This is a retrospective review of 35 Sudanese patients with confirmed cystic fibrosis. About 60% of cases presented before the age of 5 years and male to female ratio was 1.7:1.0. Consanguinity was reported in 25 of the families. The main presenting features were productive cough, wheeze and clubbing. The chest X-ray showed variable degrees of hyperinflation, collapse, cystic, fibrotic changes and bronchiectasis involving both upper and lower lobes with blurring of cardiac border and hilar vasculature in the majority of cases. The sweat chloride was between 70 and 140 mmol/l in 83% of the patients (positive > 60 mmol/l). Three patients underwent DNA study and confirmed to have cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. Gene study was not available for the rest of the patients. To our knowledge this is the first report of confirmed cases of cystic fibrosis in Sudanese patients.

Jaw osteomyelitis as a complication in osteopetrosis.

Osteopetrosis is a rare bone dysplasia. The disease is characterized by osteoclast dysfunction, producing diffuse symmetrical increase in skeletal bone density and exhibiting various clinical manifestation because of heterogeneous entity. Among them, jaw osteomyelitis, frequently mandibular osteomyelitis, is an important complication encountered in these patients. In this article, 2 patients diagnosed with osteopetrosis with mandibular osteomyelitis would be presented. We used debridement and decortications, removal of hopeless teeth, and topical phenytoin in the management of mandibular osteomyelitis.

Juvenile-onset Parkinsonism as a result of the first mutation in the adenosine triphosphate orientation domain of PINK1.

BACKGROUND: Mutations in the PTEN-induced putative kinase 1 (PINK1) gene at 1p36 have been involved in autosomal recessive early-onset parkinsonism. OBJECTIVE: To describe the clinical and genetic features of the largest kindred reported to date with early-onset parkinsonism associated with the PINK1 gene. DESIGN: Clinical and genetic study. SETTING: Collaborative study. Patients Eight patients from Sudan with particularly early onset (ages 9-17 years) and phenotypes varying from dopa-responsive dystonia-like to typical early-onset parkinsonism. MAIN OUTCOME MEASURES: The PINK1 genotype and Parkinson disease status of all available family members. RESULTS: The disease was caused by a novel mutation, p.A217D, located in the highly conserved adenosine triphosphate orientation site of the PINK1 kinase domain. CONCLUSION: This study extends the phenotypic and molecular spectrum of the PINK1 gene and the geographic origin of patients with PINK1 gene mutations.