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.     

Common environmental allergens causing respiratory allergy in India

Respiratory allergy affects all age groups but the children are the worst affected by the respiratory allergy. Bioparticles from different biological sources are the main cause of allergy. Pollen grains, fungal spores, insect and other materials of biological origin form the most important allergen load in the air. For the efficient diagnosis of the allergy and its effective treatment it is very important to know about the prevalence, seasonal and annual variations of aeroallergens of the area. India being the climatically diversed country, there is diversity in the flora and fauna of different parts of the country. Atmospheric surveys carried out in different parts of India reveal that,Alanus nitida, Amarantus spinosus, Argemone mexicana Cocos nucifera, Betula utilis, Borasus flabellifer, Caraica papaya, Cedrus deodara, Cassia fistula, Parthenium, Chenopodium album,Dodonaea viscosa, Malotus phillipensis, Plantago ovata, Prosopis juliflora, Ricinus communis, Holoptelea intergifolia are the allergenically important pollens of the country. Among the fungal aeroallergens,Altemaria, Candida aibieans, Aspergillus versicolor, Aspergillus terreus, Aspergillus japonicus, Cladosporium cladosporoides, Fusarium roseum, Ganoderma lucidum,Neurospora sitophila Helminthosporium, Ustilago trtici, Uromyses are important allergens. Dust mitesD. farinae,D.pteronyssinus are also important source of inhalant allergens particularly in the coastal areas of the country. Cockroaches, beetles, weevils, mosquitoes, house flies also contribute towards the aeroallergen load and are allergenically implicated. Avoidance of the indoor and outdoor aeroallergens is recommended for better management of respiratory allergy.     

von Hippel-Lindau disease with bilateral multiple renal cell carcinoma managed by right radical nephrectomy and left repeat partial nephrectomy

von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by cysts and cystadenoma in the kidney, pancreas and epididymis and angiomas of the central nervous system and retina as well as renal cell carcinoma (RCC), phaeochromocytoma, islet tumors of the pancreas, and endolympatic sac tumors. VHL for its multicentric-characteristic and bilateralism often puts the surgeon in challenging situation. We present a case of VHL with bilateral RCC and retinal angiomas managed with right radical nephrectomy and left repeat partial nephrectomy.     

Pseudohypospadias with bilateral vesicoureteric reflux: consequences of inappropriate management of stricture urethra

A 17-year-old male boy presented with clinical features of pseudohypospadias (small penile stump with absent distal penile urethra) associated with very small capacity bladder and bilateral grade IV vesicoureteric reflux following suprapubic urinary diversion for multiple urethro-cutaneous fistula and periurethral abscess which developed as a consequence of inappropriate initial management of urethral trauma. This case highlights the importance of the initial management of urethral trauma and the management of its rare complication.     

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.