Singap Med J
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The quality of life of transfusion-dependent thalassaemia patients is affected by the disease itself and iron overload complications from repeated blood transfusion. Desferrioxamine has been used to remove the excess iron, resulting in decreased mortality and morbidity. In Malaysia, a significant proportion of the transfusion-dependent thalassaemia patients are not prescribed desferrioxamine, due to its high cost, especially as it is not subsidized by the government. The aim of this study was to measure the quality of life of thalassaemia patients on desferrioxamine treatment. ⋯ Optimum desferrioxamine usage reduces iron overload complications and provides a better quality of life.
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Micropenis may be an important sign of underlying hypogonadism or pituitary hypofunction in the neonatal period. Penile lengths of normal newborns have been reported in many Western populations. However, the data may not be applicable in the Asian or the multiracial Malaysian population. Our study aimed to establish the normal penile length and testicular volume in term newborn infants in the major ethnic groups in Malaysia. ⋯ Using -2.5 standard deviations as the cut-off for micropenis, a Malay or Chinese newborn infant in Malaysia with a penile length of less than 25 mm is considered to have a micropenis and further evaluation is warranted.
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Case Reports
Grade 4 spondylolisthesis of the L5 vertebra associated with dural ectasia in neurofibromatosis.
Spondylolisthesis associated with neurofibromatosis is rare, and only 12 cases have been reported so far. However, only one report of grade 4 spondylolisthesis with neurofibromatosis has been reported in the literature. A 15-year-old boy with neurofibromatosis was admitted for back pain and neurological claudication. ⋯ The patient was re-operated on, to provide additional stability with pelvic fixation. He was pain-free with a good fusion at the two-year follow-up. Adequate posterior stabilisation with fusion gives good results in grade 4 spondylolisthesis associated with neurofibromatosis and dural ectasia.
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Ingestional naphthalene mothball poisoning leading to prolonged haemolysis and methaemoglobinaemia can present with diagnostic and therapeutic challenges. A 19-year-old woman ingested 12 mothballs, and presented two days later with haemolysis and methaemoglobinaemia. She was treated with red blood cell transfusions, intravenous methylene blue, N-acetylcysteine and ascorbic acid. ⋯ Haemolysis with anaemia and methaemoglobinaemia persisted even after five days post-ingestion. Clinical and biochemical parameters improved. We describe a case of ingestional naphthalene poisoning with a good outcome after treatment.
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Necrotising fasciitis is a life-threatening soft tissue infection that is associated with high mortality and morbidity. It has been described in the form of Fournier's gangrene following rectal perforations related to colorectal cancer. In these rare instances, spontaneous perforation of locally-advanced rectal carcinoma provides an entry point for bacterial seeding to the surrounding soft tissues, resulting in Gram-negative sepsis of the perineum. ⋯ We report an unusual self-induced traumatic rectal perforation presenting with severe necrotising fasciitis of the lower limb in a 73-year-old Chinese man. Our patient was successfully treated with a multidisciplinary approach that involved a defunctioning colostomy as well as prompt and rigorous debridement of the affected limb. We also review the literature on the management of retroperitoneal rectal perforations and their sequela, as well as discuss the various surgical options commonly applied and their outcomes.