Multicystic nephroma masquerading as hydatid cyst: a diagnostic challenge.

BACKGROUND: Multicystic nephroma is an uncommon, non-familial renal neoplasm that is usually benign. About 200 cases of this lesion have been described in the literature. CASE PRESENTATION: We report on a Sudanese child who presented at the age of two and a half years with an abdominal mass, clinical and radiological features favored the diagnosis of hydatid cyst which is endemic in this African tropical country, and the diagnosis of multicystic nephroma was only possible after histopathological examination. CONCLUSION: Multicystic nephroma is a rare benign tumour with an excellent prognosis. Clinical and radiological differentiation of multicystic nephroma from hydatid cyst is difficult. Thus, histopathological examination of the surgical specimens seems to be the only feasible method of making the correct diagnosis.

Osteitis of the radius following Bacillus Calmette-Guérin vaccination at birth: a case report.

BACKGROUND: The Bacillus Calmette-Guérin vaccine, which is used for the prevention of tuberculosis, is considered protective against the severe forms of childhood tuberculosis. However, some serious adverse reactions including osteitis of the long bones can occur. CASE PRESENTATION: We report a case of an 18-month-old Sudanese girl who presented at the age of 3 months with swelling of her left forearm following Bacillus Calmette-Guérin vaccination administered at birth. Radiological and histological investigations confirmed tuberculous osteitis of the distal radius. She responded very well to antituberculous treatment with complete healing at follow-up visits. To the best of our knowledge this is the first case report of osteitis of the radius following Bacillus Calmette-Guérin vaccination described from Sudan. CONCLUSIONS: Bacillus Calmette-Guérin osteitis, although rare, should be considered a possible complication of the Bacillus Calmette-Guérin vaccination, and early diagnosis and treatment are essential.

Brain tuberculoma, an unusual cause of stroke in a child with trisomy 21: a case report.

BACKGROUND: Tuberculosis remains a public health problem in developing countries and is associated with lethal central nervous system complications. Intracranial tuberculomas occur in 13% of children with neurotuberculosis. Patients with trisomy 21 have an increased risk for stroke, which usually stems from cardiovascular defects. CASE PRESENTATION: We report a case of a 12-year-old Sudanese boy with trisomy 21 who was presented to our hospital with focal convulsions and right-sided weakness. The results of neuroimaging and histopathological examinations were consistent with cerebral tuberculoma. The patient had a good initial response to antituberculosis drugs and steroids. To the best of our knowledge, this is the first case report of multiple brain tuberculomas described in a child with trisomy 21. CONCLUSIONS: Patients with trisomy 21 have an increased risk for stroke. Our patient had an exceptional case of stroke caused by tuberculoma. The present case emphasizes the need to consider tuberculomas in the differential diagnosis of children with neurological symptoms living in areas of high tuberculosis incidence.

Prune belly syndrome: A report of 15 cases from Sudan.

Prune belly syndrome is a rare congenital malformation of unknown aetiology, composed of a triad of deficient abdominal wall muscle, cryptorchidism and urinary tract anomalies. The majority of patients have associated pulmonary, skeletal, cardiac, and gastrointestinal defects. This was a prospective, case finding study that was conducted in the main paediatric hospitals in Khartoum state, during the period December 2015 to September 2016. A total of 15 patients with prune belly syndrome were collected. Patients' characteristics were noted including socio-demographic data, laboratory and radiological investigations and any medical or surgical intervention. There were 12 males and 3 females with a male to female ratio of 4:1. Most of the patients (80%) had hydronephrosis and hydroureter. The study revealed that 60% of the patients had associated anomalies, there were 4 (26.6%) with cardiac defects, 3 (20%) with orthopaedic defects one patient with small bowel volvulus and one patient with cleft lip. 6 (40%) patients received medical intervention and 8 (53%) patients underwent surgical procedures. At the last follow up visit, 2 (13.4%) patients had normal renal function tests, 8 (53.3%) ended with chronic kidney disease, and 5 died with a mortality rate of 33.3%. Prune belly syndrome is a rare entity with wide variability in severity and clinical manifestations. The mortality in prune belly syndrome remains high despite medical and surgical interventions.

Validity and reliability of Arabic MOS social support survey.

We aimed to generate a valid reliable Arabic version of MOS social support survey (MOS-SSS). We did a cross sectional study in medical students of Faculty of Medicine in Khartoum, Sudan. We did a clustered random sampling in 500 students of which 487 were suitable for analysis. We followed the standard translation process for translating the MOS-SSS. We accomplished factor analysis to assess construct validity, and generated item-scales correlations to evaluate the convergent and discriminant validity. We extracted the Cronbach's α and Spearman Brown coefficient of spit half method to determine internal consistency. We measured stability by correlation between the scores of the MOS survey taken at two different occasions with ten days apart in 252 participants. All items correlated highly (0.788 or greater) with their hypothesized scales. All items in subscales correlated higher by two standard errors with their own scale than with any other scale. Principle component analysis with varimax rotation was conducted on the 19 items and examination of scree plot graphically suggested 4 predominant factors that account for 72 % of variance. It showed high loadings, ranging from 0.720 to 0.84 for items of emotional support, 0.699-0.845 for tangible support, 0.518-0.823 for affectionate support, and 0.740-0.816 for positive social interaction. Cronbach's alpha for overall MOS scale and subscales indicated high internal consistency. The test-retest correlation showed weak correlation between the test and retest (ranges from 0.04 to 0.104). The Arabic MOS-SSS had high validity and internal consistency.

Acute kidney injury in asphyxiated neonates admitted to a tertiary neonatal unit in Sudan.

Acute kidney injury (AKI) is a recognized complication of birth asphyxia. Early recognition of AKI is important in asphyxiated neonates as it helps in early intervention and appropriate management. The aim of this study was to determine the pattern of AKI in asphyxiated neonates and its relation to the grade of Hypoxic Ischemic Encephalopathy (HIE). This was a prospective hospital based study, conducted in the neonatal intensive care unit (NICU) at Gafaar Ibn Auf Children's Specialized Hospital during the period between January 2013 and December 2013. A total of 85 full term asphyxiated neonates who were admitted in NICU and diagnosed as HIE were enrolled in this study. with 50 (58.8%) less than 7 days of age, 31(36.5%) between (8-15) days and 4(4.7%) between (16-28) days. Males were found to be more affected than females (58.9% and 41.1%) respectively. Spontaneous vaginal delivery was the mode of delivery in 48(56.4%), assisted vaginal delivery in 14(16.5%), emergency caesarian in 19(22.4%) and elective caesarian section in. Percentage of AKI in those babies was 54.1%(46). With 30(65%) from those had non-oliguric type. Ten babies (21.7%) had serum creatinine between (1.5 - 2mg/dl), 29 (63.04%) between (2 - 3mg/dl) and 7(15.22%) between (3-4mg/ dl). This means that the majority of patients presented in injury stage. Hyperkalemia was found in (37.6%), hyponatremia in (27.1%) and hypocalceamia in (25.8%). Most of the babies with AKI had stage (ii) HIE. All babies were treated conservatively and 4(8.6%) died. In conclusion AKI was observed to be a common complication in asphyxiated neonates.

Pentalogy of Cantrell: case report and review of the literature.

Pentalogy of Cantrell is a syndrome that consists of five anomalies: a midline, upper abdominal wall abnormality; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. The pathogenesis of this condition is not fully known yet, associations are common with this condition and treatment is extremely challenging. Prognosis and outcome depends on the type of the pentalogy of Cantrell and associated cardiac anomalies. It can be diagnosed reliably by antenatal ultrasound during the first trimester. We describe a Sudanese baby of undetermined sex who had the typical features of pentalogy of Cantrell and died shortly after birth.

Apert Syndrome: Late presentation and treatment challenges.

Apert syndrome is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly of the hands and feet. Anomalies of the viscera, skeleton and cardiovascular system have also been reported… Untreated craniosynostosis leads to inhibition of brain growth and an increase in intracranial and intraorbital pressure. Most cases are sporadic, resulting from new mutations with a paternal age effect. The prognosis of Apert Syndrome depends on the severity of brain malformation and early surgical interventions. We describe a Sudanese infant with Apert syndrome who presented for the first time at the age of three months and had limited options for intervention.

The pattern of anthropometric measurements among term newborn infants in Khartoum state in relation to maternal factors.

Anthropometric measurements of neonates are an important clinical tool for diagnosis of abnormally small or large neonates. This study aimed at finding the means of the anthropometric measurements among newborn babies in Khartoum state and to relate the tendency of the anthropometric parameters to maternal and paternal factors. This observational hospital-based study was carried out in 3 hospitals in Khartoum. Birth weights, crown-heel length and head circumference were taken from term neonates and questionnaire-based interviews were conducted with the mothers. Means of the sample and their associations with maternal and paternal factors were calculated using SPSS. In addition, the means were compared to WHO- MGRS (World health organization-multi growth reference standards). It was found that the average male newborn weighed 3.16kg with a range of 2.4kg to 4.1kg, measured 48.91cm in length with a range of 46.1cm to 51.4cm and measured 34.74cm in head circumference with a range of 32.3cm to 36.9cm. As for the females, the average newborn weighed 3.11kg with a range of 2.1kg to 4.2kg, measured 48.49cm in length with a range of 45.1cm to 51.7cm and measured 34.16cm in head circumference with a range of 32.3 to 36.4. These values were found to be statistically significant when compared with the WHO- MGR. We conclude that there was a difference observed in relation to Sudanese neonate's anthropometric parameters compared to WHO-MGR standards. Growth charts specific for Sudanese neonates are urgently needed.

Infantile colic, facts and fiction.

Infantile colic is one of the major challenges of parenthood. It is one of the common reasons parents seek medical advice during their child's first 3 months of life. It is defined as paroxysms of crying lasting more than 3 hours a day, occurring more than 3 days in any week for 3 weeks in a healthy baby aged 2 weeks to 4 months. Colic is a poorly understood phenomenon affecting up to 30% of babies, underlying organic causes of excessive crying account for less than 5%. Laboratory tests and radiological examinations are unnecessary if the infant is gaining weight normally and has a normal physical examination. Treatment is limited and drug treatment has no role in management. Probiotics are now emerging as promising agents in the treatment of infantile colic. Alternative medicine (Herbal tea, fennel, glucose and massage therapy) have not proved to be consistently helpful and some might even be dangerous. In conclusion infantile colic is a common cause of maternal distress and family disturbance, the cornerstone of management remains reassurance of parents regarding the benign and self-limiting nature of the illness. There is a critical need for more evidence based treatment protocols.

Morbidity and mortality amongst infants of diabetic mothers admitted into Soba university hospital, Khartoum, Sudan.

The prevalence of diabetes during pregnancy is increasing and this is associated with an increased risk of complications in both mother and fetus. The aim of this research is to study the neonatal complications of maternal diabetes. This was a prospective observational study that was conducted in Soba university hospital between September 2010 and March 2011. All infants born to diabetic mothers during the study period were admitted to the neonatal care unit for evaluation. Data on sex, gestational age, and birth weight, mode of delivery, complications, investigations, birth injury, and length of hospital stay were recorded. Maternal data were retrieved from records. Data was analyzed using Minitab 15. A total of 50 infants of diabetic mothers (IDMs) were included in the study. Thirty infants (60%) were females and 20 (40%) were males. Forty two (84%) of the neonates were born by caesarian section, only 7(14%) were born by spontaneous vaginal delivery. Birth injury was observed in 4% of them. The mean gestational age was 37.2±2.051 weeks. The median birth weight was 3.5 kg. 14 (28%) of the babies were macrosomic, and 17 (34%) were large for gestational age (LGA). Congenital anomalies were found in 3 (6%), hypoglycemia in 6 (12%), hyperbillirubinaemia in 10 (20%), hypocalcaemia and hypomagnesaemia each occurred in 2%, transient tachypnea of the newborn occurred in 5 (10%) of the neonates and respiratory distress syndrome in 2%. Cardiomyopathy occurred in 2% and mortality was 4%. We concluded that macrosomia, LGA, and hyperbillirubinaemia were the commonest complications in IDMs, maternal glycaemic control was found to have a significant effect on a number of outcomes.

Xerophthalmia in a traditional Quran boarding school in Sudan.

PURPOSE: To determine the prevalence of xerophthalmia at a traditional boarding school where children do not receive a diet adequate in vitamin A. MATERIALS AND METHODS: A cross-sectional survey of 406 males residing in a Quranic traditional school was conducted using the World Health Organization xerophthalmia checklist. The association between the prevalence of night blindness and proportion of students staying at the school for 6 consecutive months and those eating solely at the school was investigated. The difference in age between children with night blindness and those without was investigated. Statistical significance was indicated by P<0.05. RESULTS: The prevalence of night blindness, conjunctival xerosis and Bitot's spots was 24%, 12.5% and 1%, respectively. None of the boys had corneal ulceration, corneal scars and corneal xerosis. No significant association was observed between the differences in mean age and development of night blindness (P=0.657). There was a significant association between the duration of stay (cut-off of 6 months continuously) at the institute and the development of night blindness (P=0.023). There was no statistical significance between regularly eating at the maseed and outside the "maseed" and the development of night blindness (P=0.75). CONCLUSION: Children residing at a traditional school are vulnerable to developing xerophthalmia where the diet is inadequate in vitamin A. Institutional caregivers should be made aware of the importance of providing a balanced diet rich in vitamin A. Institutional caregivers should also be educated on the signs and symptoms of vitamin A deficiency for early detection of xerophthalmia.

Meckel-Gruber syndrome: A rare and lethal anomaly.

Meckel-Gruber syndrome is a rare and lethal autosomal recessive disorder characterized by occipital encephalocele, postaxial polydactyly and bilateral dysplastic cystic kidneys. It can be associated with many other conditions. Antenatal ultrasound examination establishes the diagnosis by identifying at least two of the major features described. We describe a female baby who had the typical triad of Meckel-Gruber syndrome and died shortly after birth.

Successful separation of craniopagus conjoined twins.

Craniopagus conjoined twins represent a rare phenom- enon of congenital malformation/ dysmorphism. The clinical pathology of this complex entity is reviewed and placed in perspective. Confusion surrounds the severity of craniopagus conjoined twins especially in relation to the difficulty of separation and subsequent outcome. Successful separation of craniopagus twins remains a rarity, however modern neurosurgical tech- niques have created opportunities for successful sepa- ration and brought hope for a normal survival of these children who in the past were often left as historical footnotes or put on display as oddities of nature. We report on a craniopagus conjoined twins from Sudan who had been successfully separated.

Zellweger syndrome: A cause of neonatal hypotonia and seizures.

Zellweger syndrome, a paradigm of human peroxisomal disorders is characterized by dysmorphic features, hypotonia, severe neuro-developmental delay, hepatomegaly, renal cysts, sensorineural deafness and retinal dysfunction. This is a case report of a baby boy born with facial dysmorphism, profound hypotonia, seizures, and hepatomegaly. The diagnosis was not evident initially but only later when he presented with obstructive jaundiced and renal cysts. He died at the age of seven months. Biochemical studies revealed elevation of very long chain fatty acids and phytanic acid consistent with a peroxisomal disorder. The recognition of this syndrome is important since it is a fatal hereditary disease. Zellweger syndrome should be included in the differential diagnosis of infantile hypotonia and dysmorphism.

The sensitivity of BCG scar as an indicator of previous vaccination among Sudanese infants.

BACKGROUND: Tuberculosis remains a major cause of morbidity and mortality worldwide. Bacille Calmette Guerin (BCG) is the current vaccine for tuberculosis. The absence or presence of a scar is used as an indicator of previous vaccination. OBJECTIVES: To determine the sensitivity of the BCG scar as an indicator of previous vaccination. METHODS: We conducted a descriptive hospital based study of 100 infants attending two centres for routine vaccination and weight checks. Data analysis was done using statistical package for social sciences. RESULTS: We surveyed 100 infants, the BCG scar was present in 83%, the average size of the BCG scar was found to be 3mm. CONCLUSION: The BCG scar is a sensitive indicator of previous vaccination after the administration of the vaccine in the first month of life.