Acute burn care in resource-limited settings: A cohort study on treatment and outcomes in a rural regional referral hospital in Tanzania.

BACKGROUND: Only a few papers are published on the safety and effectiveness of acute burn care in low-income countries. A cohort study was therefore carried out to determine such outcomes. METHODS: The study was conducted in a rural Tanzanian hospital in 2017-2018. All patients admitted with burns were eligible. Complications were scored during admission as an indication for safety. Survivors of severe burn injuries were evaluated for time of reepithelialization, graft take, disability (WHODAS2.0) and quality of life (EQ5D-3L) up to 3 months post-injury, as an indication of effectiveness. RESULTS: Patients presented on average at 5 days after injury (SD 11, median 1, IQR 0-4). Three patients died at admission. The remaining 79 were included in the cohort. Their median age was 3 years (IQR 2-9, range 0.5-49), mean TBSA burned 12% (SD10%) and mortality rate 11.4%. No surgery-related mortality or life-threatening complications were observed. Skin grafting was performed on 29 patients at a delayed stage (median 23 days, IQR 15-47). Complications of skin grafts included partial (25% of procedures) and complete graft necrosis (8% of procedures). The mean time to reepithelialization was 52 (SD 42) days after admission. Disability and quality of life improved from admission to 3 months after injury (p<0.001, p<0.001, respectively). CONCLUSION: In this resource-limited setting patients presented after a delay and with multiple complications. The mortality during the first two weeks after admission was high. Surgery was found to be safe and effective. A significant improvement in disability and quality of life was observed.

The development of burn scar contractures and impact on joint function, disability and quality of life in low- and middle-income countries: A prospective cohort study with one-year follow-up.

OBJECTIVE: The aim of this study was to assess the development of burn scar contractures and their impact on joint function, disability and quality of life in a low-income country. METHODS: Patients with severe burns were eligible. Passive range of motion (ROM) was assessed using lateral goniometry. To assess the development of contractures, the measured ROM was compared to the normal ROM. To determine joint function, the normal ROM was compared to the functional ROM. In addition, disability and quality of life (QoL) were assessed. Assessments were from admission up to 12 months after injury. RESULTS: Thirty-six patients were enrolled, with a total of 124 affected joints. The follow-up rate was 83%. Limited ROM compared to normal ROM values was observed in 26/104 joints (25%) at 12 months. Limited functional ROM was observed in 55/115 joints (48%) at discharge and decreased to 22/98 joints (22%) at 12 months. Patients who had a contracture at 12 months reported more disability and lower QoL, compared to patients without a contracture (median disability 0.28 versus 0.17 (p = 0.01); QoL median 0.60 versus 0.76 (p = 0.001)). Significant predictors of developing joint contractures were patient delay and the percentage of TBSA deep burns. CONCLUSION: The prevalence of burn scar contractures was high in a low-income country. The joints with burn scar contracture were frequently limited in function. Patients who developed a contracture reported significantly more disability and lower QoL. To limit the development of burn scar contractures, timely access to safe burn care should be improved in low-income countries.

[Late diagnosis of classical galactosemia. An adult with special biochemistry]. / Zeer late herkenning van klassieke galactosemie. Een volwassene met een bijzondere biochemie.

The atypical case history of a galactosemic patient who was not recognized as such until his 22nd year is described. As in classical galactosemia there was not found any galactose-1-phosphate uridyl transferase activity in the erythrocytes. However, after the patient was on a galactose-free diet, there was no demonstrable elevation of galactose-1-phosphate in the erythrocytes. We would advocate a limited screening on galactosemia.

[Krabbe's disease: globoid cell leukodystrophy. Clinical and prenatal diagnosis]. / De ziekte van Krabbe: globoïdcel leukodystrofie. Klinische en antenatale diagnostiek.

A 9 weeks old boy with infantile globoid cell leukodystrophy is presented. Clinical features consisted of insufficient weight gain, hypertonic musculature, irritability, loss of previously attained skills, loss of visual function, elevation of the cerebrospinal fluid (CSF) protein. He died at the age of 11 months. First-trimester diagnosis was performed in two consecutive pregnancies by means of analysis of chorionic villi in the 9th week.

Dihydropyrimidine dehydrogenase deficiency leading to thymine-uraciluria. An inborn error of pyrimidine metabolism.

Three unrelated patients with excessive thymine-uraciluria due to dihydropyrimidine dehydrogenase deficiency are described. Excretory values (mmol/g creatinine) were: uracil 2.0-10.5, thymine 2.3-7.5, 5-hydroxymethyluracil 0.2-0.9. Orally administered (index patient) uracil and thymine were excreted for the greater part whilst dihydrouracil and S-dihydrothymine were mainly metabolised. Dihydropyrimidine dehydrogenase activities (nmol X h-1 X mg-1 protein) in leucocytes were 0.04, 0.01 and less than 0.01 in the patients, 0.31-1.66 in their parents, and 1.01-4.46 in controls (n = 4). The patients presented with a non-specific clinical picture of cerebral dysfunction.