Congenital dermal sinus tract in the lateral buttock: unusual presentation of a typically midline lesion

Congenital dermal sinus tracts most commonly involve the midline and may communicate with the central nervous system. We present a 3-year-old girl with a pit in the upper lateral buttock noted since birth. Surgical exploration revealed a firm tract that dissipated in the gluteal fascia, and ultrasound confirmed the absence of any deeper extension. Microscopic examination revealed a sinus tract lined by dermal appendages and surrounded by an exuberant inflammatory reaction to hair. This location is distinctly unusual, and our report expands the clinical spectrum of congenital dermal sinus tracts and raises awareness that lesions prone to having a connection with the central nervous system may present quite laterally. Furthermore, this case provides insight into the pathogenesis of a disease that is often explained as a “defect of midline closure” and also illustrates an unusual setting for so-called “pilonidal disease.”     

Rat-bite fever in children: case report and review

We report 2 cases of rat-bite fever (RBF), a multisystem zoonosis, in children and review the literature. RBF is caused by I of 2 Gram-negative organisms: Streptobacillus moniliformis or, less commonly, Spirillum minus. Both of our cases developed in school-aged girls with a history of rat exposure who presented with a multisystem illness consisting of fever, petechial and purpuric rash, arthralgia and polyarthritis. Both responded promptly to antibiotic treatment. An additional 10 cases from a MEDLINE review (1960-2000) are reviewed. RBF must be included in the differential diagnosis of febrile patients with rashes and a history of exposure to rats.     

Characterization of Swine Influenza A(H1N2) Variant,Alberta, Canada, 2020

Influenza strains circulating among swine populations can cause outbreaks in humans. In October 2020, we detected a variant influenza A subtype H1N2 of swine origin in a person in Alberta, Canada. We initiated a public health, veterinary, and laboratory investigation to identify the source of the infection and determine whether it had spread. We identified the probable source as a local pig farm where a household contact of the index patient worked. Phylogenetic analysis revealed that the isolate closely resembled strains found at that farm in 2017. Retrospective and prospective surveillance using molecular testing did not identify any secondary cases among 1,532 persons tested in the surrounding area. Quick collaboration between human and veterinary public health practitioners in this case enabled a rapid response to a potential outbreak.     

Relationship between disease severity and folate status of children with sickle cell anaemia in Enugu,South East Nigeria

BackgroundRepeated crises in children with sickle cell anaemia (SCA), which is a manifestation of disease severity, results in depletion of their minimal tissue folate stores, with higher likelihood of folate deficiency. The study aimed to determine the relationship between disease severity and the folate status of children with SCA attending University of Nigeria Teaching Hospital (UNTH), Enugu.MethodsThis was a hospital based, cross-sectional study conducted between September 2018 and March 2019. One hundred participants were recruited, consisting of 50 children having sickle cell crisis and 50 age and gender matched haemoglobin AA genotype controls. Relevant information was documented using a pretested questionnaire. Sickle cell severity score was determined using frequency of crisis, admissions and transfusions in the preceding one year, degree of liver and splenic enlargement, life-time cummulative frequency of specific complications of SCA, leucocyte count and haematocrit.ResultsFolate deficiency was observed in eight percent of the subjects and none of the controls. The difference was not significant (Fisher''s exact = 4.167, p=0.117). The odds of being folate deficient was 8.5 times more likely during anaemic crisis than in vaso-occlusive crisis, though not significant (95% C.I 0.05 – 89.750, p = 0.075). The mean SCA severity score was 8.06 ± 3.64, signifying a moderate SCA severity in the study population. There was a no relationship between folate status and severity of SCA (Fisher''s exact = 0.054, p = 0.949)ConclusionFolate status in children with SCA is not affected by their disease severity. Therefore, there may be no need for additional folate supplementation with increasing severity of sickle cell anaemia.     

Acute renal failure in Nigerian children: Port Harcourt experience

Acute renal failure (ARF) is a significant cause of morbidity and mortality in children. It may be pre-renal, intrinsic, or post-renal (obstructive) in aetiology. ARF was investigated in children in the south-southern part of Nigeria to determine the prevalence, aetiology, management and outcome of ARF. A retrospective review of data from all children from birth to 16 years of age admitted into the Department of Paediatrics, University of Port Harcourt Teaching Hospital (UPTH), with the diagnosis of ARF over an 18 year period (January 1985 to December 2003) was performed. Information was obtained about the age, sex, clinical features, blood pressure, laboratory and radiological investigations, aetiology, and treatment received including dialysis. Information on the outcome, factors influencing outcome, and possible causes of death were reviewed. There were 211 patients, 138 (65.4%) males and 73 (34.6%) females (M:F, 1.9:1), with a hospital prevalence of 11.7 cases/year. The patients were aged 5 days to 16 years (mean 5.6±4.7 years). Oliguria was the most common clinical presentation in 184 (87.2%) patients. Hypertension was seen in only 39 (18.5%) patients. The causes were age-related. The neonates had ARF from severe birth asphyxia 27 (35.5%), septicaemia 17 (22.4%), with tetanus 4 (5.3%) and congenital malformations 11 (14.5%). Sixty-one (28.9%) and 29 (13.7%) patients had ARF from gastroenteritis and malaria respectively. The patients with leukaemia were all more than 10 years old and had acute lymphoblastic leukaemia. Two patients (1.9%) had Burkitts lymphoma involving the abdomen and 3 patients had HIVAN. 112 (53%) patients had anaemia with a mean haematocrit of 20.25±6.9%. Dialysis was indicated in 108 patients, but only 24 patients (22.2%) had peritoneal dialysis (PD), because of financial constraints and lack of dialysis equipment. Mortality rate was 40.5%. The causes of death were uraemia 60 (70.6%), overwhelming infection 5 (5.9%), and recurrent anaemia 20 (23.5%). Hypertension (X² 15.7, P<0.001) and lack of dialysis (X² 7.96, P<0.01) significantly affected outcome. Other factors associated with demise were delayed presentation (58.8%), use of herbal treatment (35%), and unaffordability of treatment (40%). ARF is a significant cause of mortality in Nigerian children. The patients are not adequately managed because of poverty and lack of facilities for dialysis. The causes of ARF in our environment are preventable, and should be expected.     

Chronic renal failure in children: a report from Port Harcourt,Nigeria (1985–2000)

A 15-year review of children who presented with chronic renal failure (CRF) to the University of Port Harcourt Teaching Hospital, Rivers State of Nigeria, was carried out. Forty-five children (28 boys, 17 girls) with CRF, defined as a glomerular filtration rate below 30 ml/min per 1.73 m(2) body surface area or a rise in serum creatinine above 120 micro mol/l for at least 6 months, often accompanied by other biochemical abnormalities, were identified. The median annual incidence of CRF was 3.0 per million children. The prevalence of CRF increased from 12.5 in the 1985-1990 periods to 15 per million children after 1995. Acquired disorder was the major cause of CRF. Glomerulopathies were the cause in 53.3% of patients, mainly chronic glomerulonephritis (56.5%) and nephrotic syndrome (30.4%). Hepatitis B surface antigen was positive in 2 patients. Congenital disorders accounted for 28.9% of all cases of CRF, which is lower than data from other countries. Posterior urethral valve was the only congenital disorder causing CRF in the study. No child with hereditary renal disorder as a cause of CRF was identified. Children with congenital disorders were diagnosed at an earlier age. The mortality rate was high (46.7%), as most patients were managed conservatively, since there were no permanent facilities for chronic dialysis or renal transplantation in Nigeria. The study shows that CRF is common in Nigerian children, and there is an urgent need for the establishment of facilities for renal replacement therapy.     

Folate levels in children with sickle cell anaemia on folic acid supplementation in steady state and crises at a tertiary hospital in Enugu,Nigeria: a prospective,comparative study

IntroductionFolic acid supplementation is an integral aspect of the management of children with sickle cell anaemia (SCA) especially in Africa. In spite of this, there have been concerns about lower folate levels, especially during crisis.AimTo determine red cell folate levels of children with sickle cell anaemia in steady state and during crisis and compare with those with haemoglobin AA genotype.MethodThis study was prospective, hospital based, and comparative. Fifty children with sickle cell anaemia were recruited during crises and followed up until they met the criteria for attaining steady state. The controls were fifty children matched with those with SCA for age and gender and had haemoglobin AA genotype. Red cell folate estimation was done with the Electrochemiluminescence Immunoassay (ECLIA) method using the automated Roche Cobas e411 equipment.ResultsThe median (IQR) red cell folate level in children during sickle cell crisis was 265.95 (134.50) ng/ml, which was significantly lower than the median (IQR) of 376.30 (206.85) ng/ml obtained during steady state. Most children with SCA (41 out of 50) had significantly higher folate levels during steady state (T=1081, Z-score= -4.660, p < 0.001). Median level of red cell folate was lower during anaemic crisis compared to vaso-occlusive crisis, though not significantly so (N(50), U = 214.00, Z-score= -1.077, p = 0.305). The median red cell folate level of normal controls was 343.55 (92.90) ng/ml, which was significantly lower than the 376.30 (206.85) ng/ml obtained during steady state (N(50), U= 209.00, Z-score= -7.177, p <0.001).ConclusionMedian red cell folate levels of the study participants were within normal limits, though most children with SCA had significantly higher levels during steady state compared to crisis. Normal controls had significantly lower red cell folate levels than the children with SCA during steady state.     

Effects of MPTP on the cerebrovasculature

The neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, has been shown to cause pooling of blood in the brain microvasculature and decrease the permeability of the blood-brain barrier. All areas of the brain examined in this study were affected. This study points out the possibility that reduced nutrient uptake, hypoxia and ensuring free radical damage are involved in the mechanism of toxicity of this neurotoxin.