In human leukocytes and fibroblasts beta-mannosidase activity has a unimodal pH optimum (4.0-4.5). Markedly reduced activity is found in I-cell disease. Normal activities in human fibroblasts are ten times higher than in the goat, in which species a deficiency disease has been described.

Background: Acute confusional state or delirium is a frequent cause of hospital admission in the elderly. It is characterized by an acute fluctuating impairment of cognitive functions and inattention. Recognition and prompt treatment is crucial to decrease the morbidity and mortality of hospitalized elderly patients. Aim of this study was to evaluate the distribution of common medical conditions related to acute confusional state among elderly hospitalized Patients. Methods: This was a cross sectional study. We enrolled 380 patients from different medicine wards in Dhaka Medical College Hospital. We included patients with acute confusional state/delirium of less than 7 days duration. The enrolled patients fulfilled the diagnostic criteria of an acute confusional state. A predesigned checklist was used for data collection. Head injury was excluded by history and CT scan of brain. Patients with preexisting illnesses, such as dementia, psychiatric illnesses and recurrent seizures, and any case of poisoning was excluded. All findings were noted and recorded. A written informed consent was taken from the attendant and relatives. Result: Among 380 patients, most of confusional state developed after the age of 65 years and the mean age was 69 +/-7.6 years. Associated medical conditions were uncontrolled hypertension (63.2%), fever & infection (41.6%), uncontrolled DM (36.6%), CVD (28.4%), CKD & electrolytes abnormalities (10.5%), joint diseases & pain (13.7%). Most of the patients used plyphormacy (76.6%) out of which antihypertensive (60%) were common. The mean duration of presentation was 6.1+/-0.6 days, and among all patients about 63.7% were improved, 29.50% was in persistant symptom of confusion and 6.8% of them died. Conclusion: Acute confusional state was common after 65 years. Uncontrolled hypertension, fever & infection, uncontrolled diabetes, CVD, CKD & electrolytes abnormalities and polypharmacy were found commonly in patients with acute confusional state. J MEDICINE JAN 2021; 22 (1) : 41-45

Thrombotic thrombocytopenic purpura is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large vWF multimers and the cleaving protease ADAM TS 131 (A disintegrin and metalloproteinase with a thrombospondin type 13). This disease was first described by Moshococowitz in 1924.2 It is clinically characterized by five typical syndromes: thrombocytopenia, microangiopathic hemolytic anaemia, renal impairment, fluctuating neurological abnormalities and fever. It is a rare disease and fatal in 50 to 80 per cent of cases5. TTP can be idiopathic or secondary. Here we are describing a case who developed fever, purpuric spot, jaundice, renal impairment and fluctuating neurological features. Initially it seems to us as a case of DIC, but later hematological evaluation reveals it as a case of TTP, as the patient fulfills the pentad criteria. Unfortunately we could not do the serum ADAM TS 13 level because of lack of facility in our country and in abroad it is costly.Bangladesh J Medicine Jan 2015; 26 (1) : 35-38

Gitelman'?s syndrome, discovered in 1966 by Gittleman,Graham and Welt, is an autosomal recessive renal tubular disorder and characterized by hypokalemic metabolic alkalosis, hypomagnesaemia, hypocalciuria. This is a rare cause of hypokalemia which has an autosomal recessive inheritance. Here we are reporting a case of a 35 years old lady presented with recurrent attacks of quadriparesis due to hypokalemia and pain in multiple joints. Later we diagnosed the case as Gitelman?'s syndrome.DOI: http://dx.doi.org/10.3329/jom.v16i1.22407 J MEDICINE 2015; 16 : 59-60