Severe lower limb defects in exstrophy of the cloaca

We present here a patient with exstrophy of the cloaca associated with severe lower limb defects. The limb malformations include, on the right, a split foot with distal separation of the tibia and fibula, and on the left, attachment of the lower half of the left leg with a two-toed foot at nearly a right angle to the mid left thigh. A review of the literature indicates that 17-26% of patients with cloacal exstrophy also have lower limb defects. We hypothesize that cloacal exstrophy and associated lower limb defects have a related pathogenesis and that both are related to deficiencies of caudal mesoderm or mesodermal differentiation signals. More cases of exstrophy of the cloaca with limb defects need to be reported to better characterize the limb anomalies and to more precisely determine their frequency.     

Sorbitol Intolerance in Adults

Sorbitol is a commonly used sugar substitute in "sugar-free" food products. Although sorbitol intolerance manifested by abdominal pain, bloating, and diarrhea has been observed in children, it has not been well documented in adults. Forty-two healthy adults (23 whites, 19 nonwhites) participated in this study. After ingestion of 10 g of sorbitol solution, end expiratory breath samples were collected at 15-min intervals for 4 h and analyzed for H2 concentration. Clinical sorbitol intolerance was detected in 43% of the whites and 55% of the nonwhites, the difference not being statistically significant. However, severe clinical sorbitol intolerance was significantly more prevalent in nonwhites (32%) as compared to whites (4%). There was a good correlation between the severity of symptoms and the amount of hydrogen exhaled. Dietetic foods, many of them containing sorbitol, are very popular with diabetics and "weight watchers." Based on our observations, we believe that a large number of adults could be suffering from sorbitol-induced nonspecific abdominal symptoms and diarrhea. These symptoms could lead to an extensive diagnostic work-up and lifelong diagnosis of irritable bowel syndrome.     

Transport of molecules across tumor vasculature

The vascular-extravascular exchange of fluid and solute molecules in a tissue is determined by three transport parameters (vascular permeability, P, hydraulic conductivity, L_p, and reflection coefficient, ); the surface area for exchange, A; and the transluminal concentration and pressure gradients. The transport parameters and the exchange area for a given molecule are governed by the structure of the vessel wall. In general, tumor vessels have wide interendothelial junctions; large number of fenestrae and transendothelial channels formed by vesicles; and discontinuous or absent basement membrane. While these factors favor movement of molecules across tumor vessels, high interstitial pressure and low microvascular pressure may retard extravasation of molecules and cells, especially in large tumors. These characteristics of the transvascular transport have significant implications in tumor growth, metastasis, detection and treatment.     

Macrocarposide, a new isoflavanone c-glucoside from Pterocarpus macrocarpus heart wood

Macrocarposide has been isolated for the first time as a natural product from the heartwood of PTEROCARPUS MACROCARPUS along with some known compounds. Based on its spectral and chemical evidence, it has been assigned the structure dalbergioidin-6- C-glucoside ( 1).     

Concurrent intrapericardial–pulmonary hydatidosis: an unusual multisystem echinococcosis

A 46-year-old male presented with breathlessness for a few months. He had been operated twice for liver hydatid cysts and once for right pulmonary hydatid cysts at other hospitals. Now he was found to have one hydatid cyst in the upper lobe of the left lung and multiple hydatid cysts adjoining left heart border. On computed tomography (CT) scan chest and echocardiography, it was difficult to ascertain whether these cysts were pulmonary or intrapericardial. Left ventricular ejection fraction (LVEF) was 25%. Enzyme-linked immunosorbent assay (ELISA) was positive for hydatid. Left posterolateral thoracotomy revealed dead hydatid cyst in upper lobe of the lung that was removed. Infected mother hydatid cyst was encountered inside pericardial sac. Scores of daughter hydatid cysts, varying in size from 1 to 30 mm, were scooped out intact from the pericardial cavity. There was significant improvement in cardiac activity, once the tamponade effect of hydatid cyst was removed. Pericardium was about 1 cm thick with lot of purulent and necrotic slough. To prevent future constrictive pericarditis, subtotal pericardiectomy was done. Intrapericardial hydatid cyst should be kept in mind whenever it obscures the heart border and patient has features of cardiac tamponade. Early surgical intervention may be required in these cases.     

Covid-19 Pandemic: Resumption of Orthopedic Care and Medical Education

Covid-19 is a respiratory disease caused by coronavirus 2 (SARS-CoV-2) first identified in Wuhan, China (December 2019). The disease rapidly crossed the barrier of countries, continents and spread globally. Non-pharmaceutical measures such as social distancing, face mask, frequent hand washing and use of sanitizer remained the best available option to prevent the spread of disease. OPD, IPD admissions, elective O. Ts were curtailed. Orthopedic care was only limited to emergency and semi-urgent procedures like necrotizing fasciitis, open fracture, and compartment syndrome. These measures were taken to preserve infrastructure and manpower to manage covid-19 pandemic. The children were thought to have a low susceptibility to covid-19 as compared to an adult. Deferring the patient during pandemic has led to high orthopedic disease burden, morbidity and disease-related sequelae, hence elective care must be resumed with modified hospital infrastructure. Resumption of elective/emergent orthopedic care should be slow, phasic and strategic, much similar to unlocking. Cases must be stratified depending on covid status and severity. Dedicated O.Ts with neutral/negative pressure and HEPA filter for covid positive and suspected patients are to be used. All symptomatic and suspected patients should be investigated for covid-19 by RT-PCR, blood counts and CT scan. Regional anaesthesia should be preferred to General anaesthesia. Power drill/saw/burr/pulse lavage should be minimized to avoid aerosol generation. Postoperatively continuous surveillance and monitoring to be done for covid related symptoms. Medical institutes rapidly shifted to the online mode of education. Blended learning (virtual & physical) and imparting skills have to be continued in post covid phase with equitable distribution of teaching hours to students of different years.     

Eden-Hybinette Procedure for Revision Surgery in Recurrent Anterior Shoulder Instability in Epilepsy

AimsThe purpose of this study was to analyze the clinical outcomes after Eden-Hybinette procedure for revision surgery in recurrent anterior shoulder instability in patients with epilepsy.Materials and MethodsWe retrospectively evaluated eight such patients between 2015 and 2018. Four patients had failed Latarjet/Bristow procedure and two had failed arthroscopic Bankart procedure, while two had history of both the procedures. After medical control of epilepsy, Eden-Hybinette procedure was performed in all patients. WOSI score and Rowe shoulder score was recorded preoperatively and in subsequent post-operative follow-up. A paired t test was used to analyze and compare preoperative and postoperative outcomes and was considered significant if p value was < 0.05.ResultsThe average follow-up was 30 months (range 24–48 months). There was no recurrence of shoulder instability. The mean WOSI score before surgery was 77.3 (range 70–83), which improved to 24.2 (range 19–30) at 24-month follow-up. The mean Rowe score before surgery was 11.3 (range 5–15), which improved to 81.8(range 65–90) at 24-month follow-up. The improvement in WOSI and Rowe score was found to be statistically significant [p value < 0.05]ConclusionsWe conclude that Eden-Hybinette is a useful revision procedure to manage recurrent anterior shoulder dislocation in patients with epilepsy. Optimum medical control of seizure is also an important factor in preventing recurrent shoulder instability     

Response of the ENPP1-Deficient Skeletal Phenotype to Oral Phosphate Supplementation and/or Enzyme Replacement Therapy: Comparative Studies in Humans and Mice

Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1^asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1^asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1^asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1^asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR).