Non-secretory multiple myeloma (NSMM) is a rare variant of the classic form of multiple myeloma (MM). In NSMM, no monoclonal gammopathy can be detected in serum or urine by conventional techniques, making the diagnosis more difficult. We describe a 71-year-old man who had been diagnosed and treated for granulocytic sarcoma one year prior to his recent problems of progressive low-back pain of two months duration. Skeletal X-rays showed diffuse osteolytic lesions with multiple pathological fractures but there was no monoclonal gammopathy in the serum or urine. The biopsy of the lytic lesion on the upper part of the femur showed infiltration by abnormal plasma cells. A diagnosis of NSMM was made and he was treated with chemotherapy. The early diagnostic difficulty and the challenges faced regarding the case are discussed.
Autonomic dysreflexia (AD) is not an uncommon clinical condition and it is usually detected in patients with complete spinal injuries at or above thoracic 6th vertebral level (T 6). This condition is reported to occur in 48- 60% of cases of spinal cord injury at or above the level of T6. But AD due to injury below T6 is rare. The basic mechanism is thought to be due to excessive, uncontrolled activation of sympathetic system. In the present case, we discuss a persistent AD in 55-yr-old tetraplegic patient with C5 American Spinal Injury Association (ASIA) grade A lesion due to a fall from 10 metre height. MRI examination showed C5 and C6 bi-facets fracture and dislocation with canal compromise. Wiring and fusion was performed but recurrent mucous plugging and aspiration pneumonia and urinary tract infection happened during the hospital stay. Three months later, he was re-admitted with multiple pressure sores, pneumonia, sepsis and high blood pressure. He was administered with nifedepine but the blood pressure kept fluctuating. The present study highlights how the precipitating factors like concomitant urinary tract infection, decubitus ulcers, spasticity triggered the AD attack. The knowledge of the AD and its proper diagnosis and management may be beneficial to all clinicians and the present article attempts to highlight such.
The chorea-acanthocytosis syndrome (CHAC) is a rare disorder beginning in late adolescent or adult life in association with acanthocytosis, a normal lipid profile and characterized by progressive neurological disease. The inheritance is usually autosomal recessive, although apparent sporadic and autosomal dominant instances are also known. We report here a young man who presented with choreo-athetoid movement, dystonia, tics, symmetrical axonal polyneuropathy with normal cognitive function. The subsequent peripheral blood film reveals acanthocytes > 5%. Diagnosis of neuroacanthocytosis was made.
The present cadaveric study, describes asymmetrical right cerebral hemisphere and anomalous posterior horn of the right lateral ventricle and discusses its clinical implications. The occipital lobe of right cerebral hemisphere extended more posteriorly as compared to the left and so did the posterior horn of the lateral ventricle, resulting in the thinning of the occipital cortex. Till now, the dimension and volumetric study of the lateral ventricles have been usually performed by MRI and CT scans. A gross anatomical study of such a case, is the rarest of its kind. The abnormal anatomy of the lateral ventricles may be of great academic interest regarding CSF circulation and also important for clinical, radiological and surgical interventions (Fig. 2, Ref 8). Full Text (Free, PDF) www.bmj.sk.
The case of a 44-year-old man with left parotid enlargement that was initially diagnosed as cementifying fibroma is presented. The lesion was found in the deep lobe of the parotid gland and was successfully removed. Postoperatively, the patient recovered well with intact facial nerve function and remained asymptomatic after 1 year. Subsequent histology revealed the mass to be a benign fibrous tumour. The diagnosis and management of this rare entity are discussed.
Cystic fibrosis (CF) is an autosomal recessive disease commonly found among the Caucasian population. The availability of sweat test and with increasing experience have made it possible to diagnose more cases of CF. Our first case of CF was diagnosed 16 years ago and to date we have managed sixteen cases of CF. Sixteen children were diagnosed with CF in our units at the Paediatric Institute and University Malaya Medical Centre (UMMC). They were referred with either one or all of the following symptoms: i) recurrent pneumonia, ii) bronchiectasis, iii) failure to thrive, iii) malabsorption or iv) history of meconium ileus obstruction during the neonatal period. When the clinical features suggested strongly of CF, sweat tests will be performed in duplicates and considered positive when the sweat chloride or sweat sodium was more than 60 mmol/l for both results. Seventy- two hours fecal fat excretion or stool for fat globule was performed to document malabsorption. From the year 1987 to 2003, 16 patients were confirmed to have cystic fibrosis in Malaysia by positive sweat tests. Thirteen patients were diagnosed in Paediatric Institute while the remaining three were diagnosed in UMMC. On follow-up two patients died due to severe bronchopneumonia at the age of two years old. Although once considered rare, CF should now be considered in any children with clinical presentations of recurrent chest infections, bronchiectasis, in the presence or absence of malabsoption stmptoms and in neonates with meconium ileus obstruction.
An optical scanner system, which incorporates a He-Ne laser, photodiode detectors, and a platform for placing film, was built in the laboratory. The laser system operates at the green wavelength of 543.5 nm and functions as a scanning densitometer for measurement of optical changes in a film resulting from irradiation . The central axis electron depth dose of selected electron energies 10,12 and 14 MeV were analysed using Kodak X-Omat and Kodak Extended Dose Range (EDR2) films. The Kodak X-Omat film is routinely used for high-energy electron dose distributions in radiation therapy. The electron depth-dose measured with X-Omat film was found to agree well with standard depth-dose curves in water, obtained using an ion chamber. Conversely, the recently introduced Kodak EDR2 showed an energy dependence for electron beams, the percentage depth-dose curve shifting towards the surface for 12 and 14 MeV electron beams compared to that in water.
Objective. To compare ultrasound synovial thickness of the 2nd, 3rd and 4th metacarpophalangeal joints (MCPJ) in a group of patients with proven rheumatoid arthritis (RA) and a control group of normal individuals. Materials and Methods. This is a cross-sectional study comprising 30 rheumatoid arthritis patients and 30 healthy individuals. Ultrasound scans were performed at the dorsal side of 2nd, 3rd, and 4th MCPJ of both hands in RA patients and the healthy individuals. Synovial thickness was measured according to quantitative method. The synovial thickness of RA patients and healthy individuals was compared and statistical cut-off was identified. Results. Maximum synovial thickness was most often detected at the radial side of the 2nd MCPJ and 3rd MCPJ and ulnar side of the 4th MCPJ of both hands which is significantly higher (p < 0.05) in RA patients compared to healthy individuals. With high specificity (96%) and sensitivity (90%) the optimum cut-off value to distinguish RA patients and healthy individuals' synovial thickness differs for the radial side of the 2nd and 3rd MCPJ and ulnar side of the 4th MCPJ. Conclusion. Patients with early RA appear to exhibit a characteristic pattern of synovitis which shows radial side predominance in the 2nd and 3rd MCPJ and ulnar side in the 4th MCPJ.
Study site: University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
Isolated internal iliac aneurysms are rare. We report a case of an uncommon presentation of perineal pain and tenesmus in a man caused by the pressure effects of the aneurysm. He had a successful endovascular exclusion and thrombosis of his aneurysm. On follow up of more than 3 years he remains free of all symptoms and no recurrence of the aneurysm.
We report the challenges in managing a troubled, medically ill adolescent with end-of-life issues. Our role as multi-professional service providers complemented the family's efforts to help him reconcile with himself before death. The present experience enhances understanding of the biopsychosocial aspects of care. Every child has the right to optimal care.
Traumatic diaphragmatic hernia is a well known complication of blunt trauma to the abdomen and thorax. In the acute setting, laparotomy is mandatory. In this current era, this condition can be managed with minimally invasive surgery. We hereby report a case of delayed large left diaphragmatic hernia that was repaired with a combination of laparoscopic and thoracoscopic approach.
The adults of Calochromini with male flabellate antennae were studied. Two new Calochromus Guérin-Méneville, 1833 species with flabellate antennae, C. kelantanensis spec. nov. and C. harauensis spec. nov. are described. Dumbrellia Lea, 1909 (Calochromini) is proposed as a new junior synonym of Plateros Bourgeois, 1879 (Lycinae: Platerodini). Flabellochromus Pic, 1925 is transferred to Calochromus from synonymy with Dumbrellia based on the similar shape of the pronotum. Consequently, Calochromus lamellatus Kleine, 1926, comb. nov. from Sarawak and Flabellochromus pallidus Pic, 1925, comb. nov. (=Calochromus (Flabellochromus) pallidus Pic, 1925) from Luzon are returned to Calochromus. New combinations are proposed for three Australian species previously classified in Dumbrellia: Plateros brevicornis (Lea, 1898), comb. nov. (=Calochromus brevicornis Lea, 1898), P. pilosicornis (Lea, 1898), comb. nov. (=C. pilosicornis Lea, 1898) and P. melancholica (Lea, 1921), comb. nov. Plateros barronensis nom. nov. is proposed to replace Plateros pilosicornis (Lea, 1898), a junior secondary homonym of P. pilosicornis (Blanchard, 1853) (=Lycus pilosicornis Blanchard, 1853).
Five cases of spontaneous pneumothorax were treated with video-assisted thoracoscopic surgery (VATS). These included four cases of recurrent pneumothorax and one case of persistent pneumothorax. The mean age was 33 years. The identified bullae were eliminated with either endoloop ligation (in one patient) or stapled excision (in four patients). There were no recurrences reported in a mean follow-up of 9.4 months. In conclusion, VATS offers an equally effective and less morbid alternative to open thoracotomy in the management of primary spontaneous pneumothorax.
Three East Timorese immigrants presented with signs and symptoms of heart failure in early December 1992. One of them also had features of peripheral neuropathy. Another collapsed within 24 hours of admission and attempts at resuscitation failed. The other two showed dramatic response to oral thiamine therapy. An investigation into their dietary history established gross deficiency in fresh vegetables.