Reversal of pancytopenia following kidney transplantation in a patient of primary hyperoxaluria with bone marrow involvement

Combined liver and kidney transplantation is the ideal treatment for patients with end-stage renal failure secondary to primary hyperoxaluria and systemic oxalosis, with a functioning liver providing replacement of the deficient enzyme and a functioning kidney providing the route of excretion for the oxalate crystals. Pancytopenia from bone marrow infiltration of oxalate crystals is a rare complication of primary hyperoxaluria, and its reversal following transplant has not been described. We report the first case of pancytopenia from marrow infiltration by oxalate crystals reversing following a successful kidney transplant alone. Although kidney alone transplants do not provide the best chance of survival or quality of life as compared to a combined kidney and liver transplant, a well functioning kidney transplant is able to take care of the systemic oxalate load and ameliorate, at least for a period of time, the systemic complications of oxalosis.     

Efficacy of multiwavelength light therapy in the treatment of pressure ulcers in subjects with disorders of the spinal cord: A randomized double-blind controlled trial

OBJECTIVE: To study the efficacy of multiwavelength light therapy in the treatment of pressure ulcers in subjects with spinal cord disorders. DESIGN: Randomized controlled trial. SETTING: Neurologic rehabilitation ward of a referral center in India. PARTICIPANTS: Thirty-five subjects with spinal cord injury, with 64 pressure ulcers (stage 2, n=55; stage 3, n=8; stage 4, n=1), were randomized into treatment and control groups. One subject refused consent. Mean duration of ulcers in the treatment group was 34.2+/-45.5 days and in the control group, 57.1+/-43.5 days. INTERVENTIONS: Treatment group received 14 sessions of multiwavelength light therapy, with 46 probes of different wavelengths from a gallium-aluminum-arsenide laser source, 3 times a week. Energy used was 4.5 J/cm(2). Ulcers in the control group received sham treatment. MAIN OUTCOME MEASURES: Healing of the ulcer, defined as the complete closure of the wound with healthy scar tissue, time taken for the ulcer to heal, and stage of the ulcer and Pressure Sore Status Tool score 14 days after last treatment. RESULTS: There was no significant difference in healing between the treatment and control groups. Eighteen ulcers in treatment group and 14 in control group healed completely ( P =.802). Mean time taken by the ulcers to heal was 2.45+/-2.06 weeks in the treatment group and 1.78+/-2.13 weeks in the control group ( P =.330). Time taken for stage 3 and 4 ulcers to reach stage 2 was 2.25+/-0.5 weeks in treatment group and 4.33+/-1.53 weeks in control group ( P =.047). CONCLUSIONS: Multiwavelength light therapy from a gallium-aluminum-arsenide laser source did not influence overall healing pressure ulcers. Limited evidence suggested that it improved healing of stage 3 and 4 pressure ulcers.     

Modulators of nicotinic acetylcholine receptors as analgesics

The analgesic properties of nicotine have prompted attempts to develop compounds that specifically target nicotinic acetylcholine receptors (nAChRs) in the nervous system, with the beneficial effects of nicotine but without its side effects. Thus far, only nAChR agonists have been reported as being in development for pain, although nAChR antagonists could also have a potentially analgesic action. Various problems associated with the use of nAChR agonists as analgesics have been identified and measures suggested to overcome some of them. This review describes the nAChR agonists A-85380, tebanicline, ABT-366833, ABT-202, ABT-894, epibatidine analogs and SIB-1663, of which ABT-366833, ABT-202 and ABT-894 are currently undergoing development as pain therapeutics. In vivo studies of the pathomechanism of neuropathic pain indicate that targeting alpha3beta4 does not have a specific action on neuropathic pain, and that alpha3beta4 ligands cause side effects. On the other hand, alpha4beta2 receptors are specific for neuropathic pain, and ligands that bind preferentially to these receptors both effectively relieve pain and do not cause many adverse effects. This is the basis of the difference between the action of tebanicline, which binds with greater specificity to alpha3beta4 receptors, and ABT-366833, which binds more specifically to alpha4beta2 receptors.     

Intra-individual difference between recumbent length and stature among growing children

Objective Difference between recumbent length and stature in an individual has been observed by many researchers in the past in European and American populations. The present study explores the intra-individual differences between recumbent length and stature (R.L.-Stature).Methods: A sample of 920 school going children was considered in Chandigarh city.Result: These differences are found to be statistically significant and have definite relationship with age and weight of the children. The mean difference between recumbent length and stature is found to be greater in males than in females between 5 through 10 years and between 15 through 18 years of age. However, between 10 through 15 years, it was greater in females than in males.Conclusion: This was largely due to the fact that adolescent spurt in stature preceded by two to three years in girls than that of boys. This difference was also found to be significantly correlated with age, weight and stature in both the sexes. Two regression equations are derived separately for the two sexes, from which, one can convert recumbent length into stature and vice-versa     

Stem cell technologies in regenerative medicine

The IIR Life Sciences conference on stem cell technologies in regenerative medicine was held in London, UK on 11 - 12 July 2002. The conference covered not only technologies but also ethical/regulatory and financial aspects of embryonic stem (ES) cell therapy. An excellent introduction to embryonic stem cells was given by Prof. William Kridel (Ferghana Partners, London, UK). Details of basic technologies are not described as they are covered in a detailed report on cell therapy [1]. Due to limitation of space only a selected few of the seventeen presentations are reported here.     

Benign metastasizing leiomyomatosis: case report and review

The authors report an interesting case of a minimally symptomatic 23-year-old African American woman who was found to have extensive diffuse reticulonodular opacities of the lungs on a routine chest radiograph. She had a hysterectomy 5 years previously for multiple leiomyomas of the uterus. She had no history of any prior exposure to dusts or toxins. Collagen vasculitides and bacterial, mycobacterial, and fungal infectious causes were excluded through standard testing, and a bronchoscopic lung biopsy was nonspecific. An open lung biopsy revealed multiple nodules of proliferating smooth muscle cells intermixed with irregular areas of epithelial-lined spaces. Histologically, the muscle cells appear benign with a very low mitotic rate, and the pathologic findings were consistent with benign metastasizing leiomyomatosis (BML). Staining for estrogen and progesterone receptors, actin, and c-kit were performed. This case and the review of the medical literature support the concept that BML originates from an antecedent leiomyoma of the uterus in virtually all cases with rare exceptions. It appears that tumor metastasizes to lungs or other extrauterine tissues via hematogenous spread. However, the origin of the tumor remains controversial. BML is a rare entity, with only a handful of reports in the medical literature. The authors report an interesting case of BML in a 23-year-old patient who, to their knowledge, is the youngest such patient described and who, at 13 years, has the longest period of clinical follow-up. In this article, the authors review the pathogenesis, cytogenetics, histologic markers, and management options of this rare entity.     

Pulmonary function tests in children with adenotonsillar hypertrophy

OBJECTIVE: Adenotonsillar hypertrophy, a common malady in children may lead on to cardiopulmonary dysfunction in untreated cases. The present study was designed to see alteration in pulmonary function tests i.e. spirometric parameters and blood oxygen saturation consequent to adenotonsillar hypertrophy and subsequent changes after adenotonsillectomy. METHODS: Spirometry and pulse oximetry were carried out in 40 children with adenotonsillar hypertrophy of both sexes between the age of 7 and 14 years and 40 age and sex matched healthy children. The test was repeated one and half months after surgery in study group and data was statistically analyzed using students' t-test. RESULTS: The flow volume plot was abnormal in all the patients along with hypoxia, reduced FIF50% and, increased FEF50%/FIF50%, FEV1/PEFR, FEV1/FEV0.5 ratios. There was statistically significant improvement in above parameters following surgery. CONCLUSIONS: Spirometry can prove an useful diagnostic tool in adenotonsillar hypertrophy for deciding early intervention to prevent cardio pulmonary complications.     

Histologic and Manometric Studies on the Esophagus Following Endoscopic Sclerotherapy

Objective The aim of this work was to study the histologic and manometric changes in the distal esophagus beyond 2 years following endoscopic sclerotherapy (EST) and/or surgical intervention, and to try to understand the etiological factors associated with these changes. Patients and interventions Forty patients, with an average age of 61.5 years, were studied for 2–12 years following sclerotherapy and/or surgical intervention. The causes of liver disease were alcoholic cirrhosis (78.6%), primary biliary cirrhosis (14.3%), and chronic aggressive hepatitis (7.1%). A predominant number of cases (65%) had a mesocaval interposition shunt due to the failure of EST, 32.5% EST alone, and 2.5% esophageal devascularization. All patients had esophageal manometry following mucosal biopsies taken in duplicate endoscopically from three levels of the distal esophagus. Results In the EST and shunt groups, 88.5% had manometric abnormalities, esophagitis, and chronic inflammatory changes. In the EST group, all but two patients had manometric abnormalities and chronic inflammatory changes. Analysis of the patient groups on the basis of the number of EST sessions and the amount of sclerosant injected showed that both histologic changes and dysmotility were more profound in those treated over five times with EST. The differences were significant. Conclusion It appears that EST causes persistent manometric abnormalities and chronic inflammatory changes in the distal esophagus, the severity of which seems to vary directly with the frequency of sclerotherapy and not amount of sclerosant injected.