Lysosomal acid lipase (LAL) deficiency is a rare autosomal recessive disorder which causes two distinct clinical phenotypes: Wolman's disease and cholesterol ester storage disease. LAL hydrolyses LDL-derived triglycerides and cholesterol esters to glycerol or cholesterol and free fatty acids. Its deficiency leads to accumulation of intracellular triglycerides and/or cholesterol esters. In early onset LAL deficiency, clinical manifestations start in the first few weeks of life with persistent vomiting, failure to thrive, hepatosplenomegaly, liver dysfunction and hepatic failure. Adrenal calcification is a striking feature but is present in only about 50% of cases. We report a case of an infant presenting with vomiting, diarrhoea, hepatosplenomegaly and poor weight gain that was subsequently diagnosed as Wolman's disease. He was entered into a clinical trial for LAL replacement therapy. This case reinforces that early onset LAL deficiency should be considered in a baby presenting with failure to thrive, gastrointestinal symptoms and hepatosplenomegaly.

This was a prospective, clinical study to evaluate the effect of abdominal and vaginal hysterectomy for benign indications on urinary symptoms and residual bladder volume. One hundred and seven women undergoing hysterectomy for benign conditions were included in the study that took place in a district general hospital between April 1998 and January 2000. Urinary symptoms such as stress incontinence of urine, urgency, frequency, nocturia, sensation of incomplete voiding and voiding difficulties were considered. A questionnaire was filled out and the residual bladder volume measured with a 'Bard' bladder scanner on three occasions-before the operation, postoperative days 3 or 4 and at the 6-week postoperative visit. Statistical analysis involved using a generalised estimating equation and significance assessed at the 5% level. Each woman acted as her own control. There was no evidence of changes in nocturia and voiding difficulties after surgery. All other symptoms and residual bladder volumes decreased significantly postoperatively. The type of hysterectomy did not have an effect.

This is an observational retrospective study, which included 77 patients who underwent sacrospinous fixation (SSF) over a period of 3 years in a district general hospital. Casenotes were reviewed and all patients were invited to attend a further review appointment (14-49 months). The object was to determine short- and long-term success of SSF,particularly in elderly (28% were > or = 70 years), obese (24.7%) and medically compromised (64.9%) patients. Fifty-two patients (67.5%) had previous gynaecological procedures, while the remaining 25 (32.5%) had no previous surgery. Intra- and postoperative complications were 3.9% and 32.5%, respectively. There was considerable relief of patients' symptoms at both short- and long-term followup, while the rate of recurrence of vault prolapse was 10.3%. Our experience suggests that SSF appears to be a safe and effective procedure, especially for those who may constitute surgical or anaesthetic risks. It has a reasonable success rate, good postoperative recovery and acceptable long-term results.

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During the current pandemic of Coronavirus disease (COVID)-19, multiple cases of a new condition in children have been reported with multisystem involvement associated with severe acute respiratory syndrome coronavirus-2, requiring intensive care admission. The World Health Organization has defined it as multisystem inflammatory syndrome in children and adolescents. Here, we report a similar case of a 15-year-old adolescent who presented with clinical features of Kawasaki-like illness along with multisystem involvement. The echocardiogram suggested the presence of coronary artery dilatation and the patient responded well to Intravenous immunoglobulin. Hence, pediatricians need to be aware of this condition in children with multisystemic involvement and these patients need to be treated as early as possible since they may deteriorate quickly.