Lane-Hamilton syndrome - Is it really a needle in a haystack?

Introduction: The association of pulmonary hemosiderosis with celiac disease (Lane-Hamilton syndrome) is extremely rare. Case Details: A five-year-old female child presented with fever, cough, breathlessness, and pallor for 20 days, without any previous history of recurrent lower respiratory tract infections, tuberculosis, or cardiac disease. There was no history of pica, chronic diarrhea, bleeding, or personal or family history of repeated blood transfusions. She had tachycardia, tachypnea, severe pallor, stunting, rickets, and bilateral fine lung crepitations. Peripheral smear and blood indices revealed dimorphic anemia. Anti-tissue transglutaminase IgA antibody levels were high (>200 U/mL) and the upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of celiac disease. The child was discharged on a gluten-free diet (GFD) and oral hematinic, but her dietary compliance was poor. Interestingly, the child had persistent bilateral pulmonary infiltrates, which was initially attributed to congestive cardiac failure (CCF), which persisted even despite treatment. HRCT chest revealed interstitial thickening and bilateral alveolar shadows and bronchoalveolar lavage showed a few inflammatory cells. The child was readmitted four times with similar complaints and was given packed red cell transfusions. In the fourth admission, a lung biopsy was done, which revealed extensive pulmonary hemosiderosis. The patient was given a course of oral steroids for 6 weeks, with a gluten-free diet, following which both the anemia and the pulmonary infiltrates resolved. Conclusion: Pulmonary hemosiderosis is an important cause of anemia in cases of celiac disease and may be misdiagnosed as CCF due to severe anemia. A strict GFD, with or without corticosteroids, can reverse the clinical and radiological picture.

Minimally invasive techniques as a first line approach in the management of emphysematous pyelonephritis - A single centre experience.

Context: Emphysematous pyelonephritis (EPN) is a dangerous necrotizing infection of the kidney involving the diabetics with a high case fatality rate. Recent medical literature has shown shifting of treatment strategy from conventional radical approach to minimally invasive approach. Aims: The aim of our study was to assess the role of minimally invasive stepwise decompression techniques in the management of EPN and preservation of the renal unit. Settings and Design: : This was a retrospective observational study conducted from June 2017 to April 2020 at a tertiary care centre. Material and Methods: We reviewed the hospital online records of 18 patients diagnosed with EPN for patient demographics, clinical profiles, co-morbidities, laboratory and, radiological investigations, surgical interventions performed and the outcomes. The severity of EPN was graded as per the Huang classification. Patients underwent surgical interventions as per the treatment protocol and response was assessed. Statistical Analysis Used: Descriptive statistics was applied. Results: Diabetes mellitus was present in 15 (83.3%) patients along with urinary tract obstruction in 8 (44.4%) patients. Flank pain (77.7%) was the most common presenting clinical feature while Escherichia coli (55.5%) were the most common causative organism. Most patients (50%) had Type- II EPN, all of which were managed successfully by minimally invasive procedures. In total seventeen patients (94.4%) responded well while one patient (5.5%) underwent nephrectomy with no mortality. Conclusions: Renal salvage in EPN requires multidisciplinary approach including the initial medical management followed by properly selected stepwise decompressive surgical techniques. Conservative management and decompression techniques have shown to improve patient's outcome, reducing the traditional morbidity associated with nephrectomy.

The evolution of artificial hair implantation.

From the beginning, artificial hair implantation has aroused a lot of interest, sometimes controversial, in the field of hair restoration. The artificial fiber must be of high quality and biocompatible. Biofibre® is a very fine fiber having diameter of 80 m. The standard length of this fiber is 15 or 30 cm, to satisfy multiple patient requests. They are available in 13 colors and 3 different shapes (straight, wavy and curly). Artificial hair implant is indicated in all (male and female) cases of androgenetic alopecia, especially for those who show dismal response to medical therapies and have poor donor area supply for hair transplantation. It has also shown good results to treat scars. One hundred thirty-three cases of androgenetic alopecia were treated with Biofibre® and followed-up for 3 years. A clinical evaluation was carried out after 1 month, 4 months and every other 4 months after the implant. The fiber loss was no more than 10% per year in 91.4% of the cases, 15% in 7.8% of the cases and 20% in 0.8% of the cases. 96.2% of patients declared themselves to be satisfied with the result of the implant while 3.8% declared to be not satisfied. To conclude, modern artificial hair implantation can be considered a safe and easy mode of hair restoration, for male and female patients, especially in donor depleted cases or when an immediate aesthetic result is required.

When potion becomes poison! A case report of flecainide toxicity.

We report a 48-day-old female infant, who developed cardiac conduction abnormalities and seizures secondary to supratherapeutic doses of oral flecainide. Flecainide was started in this infant for treatment of supraventricular tachycardia. The drug was withdrawn with successful normalization of the QRS complex and no further recurrence of seizures. The Naranjo probability score for adverse drug reaction was 8, making the causality "probable." The case restates an important message that physicians should be aware of the side effects of the drugs that they prescribe, especially of those drugs which have a narrow therapeutic window.

"FISHed" out the diagnosis: A case of DiGeorge syndrome.

Our patient presented with congenital heart disease (CHD: Tetralogy of Fallot), hypocalcemia, hypoparathyroidism, and facial dysmorphisms. Suspecting DiGeorge syndrome (DGS), a fluorescence in situ hybridization (FISH) analysis for 22q11.2 deletion was made. The child had a hemizygous deletion in the 22q11.2 region, diagnostic of DGS. Unfortunately, the patient succumbed to the heart disease. DGS is the most common microdeletion syndrome, and probably underrecognized due to the varied manifestations. This case stresses the importance of a detailed physical examination and a high index of suspicion for diagnosing this genetic condition. Timely diagnosis can help manage and monitor these patients better and also offer prenatal diagnosis in the next pregnancy.

Progressive Disseminated Histoplasmosis with Coomb's Positive Hemolytic Anemia in an Immunocompetent Host.

Progressive Disseminated Histoplasmosis (PDH) is mainly described in immuno-compromised individuals and rare in immuno-competent subjects. Here we report a case of progressive disseminated histoplasmosis with Comb's positive hemolytic anemia, which is infrequently reported from a country like India where histoplasmosis is not an endemic mycosis.

Sphincter of Oddi disorder (SOD): Is it necessary to investigate and treat?

Sphincter of Oddi disorder (SOD) is a part of functional gastrointestinal disorder which is a non-calculous obstructive disorder. This disease is more common in middle-aged women with a prevalence of around 1.5% but in patients with post-cholecystectomy syndrome (PCS) the prevalence rate is markedly higher (9-55%). This high variability maybe attributed to lack of uniformity in patient selection criteria, definition of SOD, and the diagnostic method used. Abdominal pain is the most common symptom occurring due to obstruction at the SO leading to ductal hypertension, ischemia from spastic contraction and hypersensitivity of papilla. Clinical diagnosis of SOD can be achieved by Rome III criteria. Various classifications are used (Milwaukee billiary and modified Milwaukee group classification) for billiary and pancreatic SOD. Not a single non-invasive method is diagnostic. Sphincter of Oddimanometry (SOM) is the gold standard method for evaluating and deciding the management of an SOD patient. The symptomatic relief rate varies from 55% to 95%, so risk-benefit ratio should be evaluated with each patient.

Steady state temperature distribution in dermal regions of an irregular tapered shaped human limb with variable eccentricity.

The investigators in the past have developed some models of temperature distribution in the human limb assuming it as a regular circular or elliptical tapered cylinder. But in reality the limb is not of regular tapered cylindrical shape. The radius and eccentricity are not same throughout the limb. In view of above a model of temperature distribution in the irregular tapered elliptical shaped human limb is proposed for a three dimensional steady state case in this paper. The limb is assumed to be composed of multiple cylindrical substructures with variable radius and eccentricity. The mathematical model incorporates the effect of blood mass flow rate, metabolic activity and thermal conductivity. The outer surface is exposed to the environment and appropriate boundary conditions have been framed. The finite element method has been employed to obtain the solution. The temperature profiles have been computed in the dermal layers of a human limb and used to study the effect of shape, microstructure and biophysical parameters on temperature distribution in human limbs. The proposed model is one of the most realistic model as compared to conventional models as this can be effectively employed to every regular and nonregular structures of the body with variable radius and eccentricity to study the thermal behaviour.

Responses of Beta vulgaris exposed to cadmium and zinc through soil drenching.

The present study investigates the responses of Indian palak (Beta vulgaris L. cv. All Green H1) exposed to cadmium (Cd) and zinc (Zn) at growth, biochemical and physiological levels. The results revealed that Cd and Zn accumulation was higher in shoots as compared to the roots of B. vulgaris plants. The increased application rates of Zn in combination with Cd significantly reduced the accumulation of Cd in below and above ground parts of B. vulgaris, whereas it increased Zn accumulation. Treatments of B. vulgaris with Cd and Zn individually or in combination significantly reduced the leaf area, biomass, photosynthetic pigments, photosynthetic rate, Fv/Fm ratio and protein contents at p < 0.05. Contrary to this, lipid peroxidation, ascorbic acid, proline and thiol contents and peroxidase activity increased significantly as compared to control at p < 0.05. The results also revealed that the combined effectof Cd and Zn were more pronounced at higher concentrations as compared to other treatments. Thus, the present study suggests that Zn may be applied to Cd contaminated field to reduce Cd accumulation in plants. However, finding of a suitable dose and toxicity level of Zn must be worked out further its application.

Direct measurement of magnon temperature: new insight into magnon-phonon coupling in magnetic insulators.

We present spatially resolved measurements of the magnon temperature in a magnetic insulator subject to a thermal gradient. Our data reveal an unexpectedly close correspondence between the spatial dependencies of the exchange magnon and phonon temperatures. These results indicate that if--as is currently thought--the transverse spin Seebeck effect is caused by a temperature difference between the magnon and phonon baths, it must be the case that the magnon temperature is spectrally nonuniform and that the effect is driven by the sparsely populated dipolar region of the magnon spectrum.

Influence of Early Life Factors, including breast milk Composition, on the Microbiome of Infants Born to Mothers with and without Inflammatory Bowel Disease.

BACKGROUND AND AIMS: Herein we analysed the influence of early life factors, including breast milk composition, on the development of the intestinal microbiota of infants born to mothers with and without IBD. METHODS: The MECONIUM [Exploring MEChanisms Of disease traNsmission In Utero through the Microbiome] study is a prospective cohort study consisting of pregnant women with or without IBD and their infants. Longitudinal stool samples were collected from babies and analysed using 16s rRNA sequencing and faecal calprotectin. Breast milk proteomics was profiled using Olink inflammation panel. RESULTS: We analysed gut microbiota of 1034 faecal samples from 294 infants [80 born to mothers with and 214 to mothers without IBD]. Alpha diversity was driven by maternal IBD status and time point. The major influencers of the overall composition of the microbiota were mode of delivery, feeding, and maternal IBD status. Specific taxa were associated with these exposures, and maternal IBD was associated with a reduction in Bifidobacterium. In 312 breast milk samples [91 from mothers with IBD], mothers with IBD displayed lower abundance of proteins involved in immune regulation, such as thymic stromal lymphopoietin, interleukin-12 subunit beta, tumour necrosis factor-beta, and C-C motif chemokine 20, as compared with control mothers [adjusted p = 0.0016, 0.049, 0.049, and 0.049, respectively], with negative correlations with baby´s calprotectin, and microbiome at different time points. CONCLUSION: Maternal IBD diagnosis influences microbiota in their offspring during early life. The proteomic profile of breast milk of women with IBD differs from that of women without IBD, with distinct time-dependent associations with baby's gut microbiome and feacal calprotectin.

An unusual cause of central cyanosis in a nine-year-old boy.

Pulmonary arteriovenous malformations are rare vascular anomalies which rarely present in childhood. We discuss a nine-year-old male child who presented with the classical triad of exertional dyspnea, cyanosis and clubbing since six years of age. Pulmonary arteriovenous malformation was suspected on chest radiography and was later confirmed on computed tomography scan. A bubble contrast echocardiography showed the presence of an extracardiac shunt. Pulmonary angiography was done to delineate the anatomy of the pulmonary vasculature and to plan embolization. The child has undergone two sittings of transcatheter coil embolization with improvement in oxygenation.

Psychedelic-assisted psychotherapy to treat psychiatric and existential distress in life-threatening medical illnesses and palliative care.

Psychiatric and existential distress commonly occur in advanced cancer and other serious, life-threatening or end-of-life medical illnesses and are associated with poor medical and psychiatric outcomes. Currently available treatment modalities in this patient population, including medication and psychotherapy, are limited in effectiveness, especially regarding existential distress. The lack of effective psycho-spiritual interventions is a critical shortcoming in palliative care and represents a high unmet need in medicine. In this commentary, we review the rationale of researching and developing psychedelic-assisted psychotherapy as a novel pharmacologic-psychotherapeutic intervention to treat psychiatric and existential distress in life-threatening medical conditions and palliative care. This paper reviews efficacy data from first and second waves of psychedelic research, and future directions for research and implementation science. More rigorous research, especially funded by governments, is needed to assess effectiveness and mechanisms of action of psychedelic therapies to treat psychiatric and existential distress in life-threatening medical illnesses and palliative care. If psychedelic-assisted treatments were made available as approved and prescribable medications in people with serious medical illnesses, it could be a significant development that opens up a pathway for clinical dissemination and public health impact internationally.

Nonparasitic splenic cysts in children: Current status.

BACKGROUND: at present, the management of nonparasitic splenic (NPS) cysts in children is not well described in literature. The aim of this study was to evaluate the current status of NPS cysts in the world literature and develop some higher level of evidence in the management of this disease entity. In this study, we have also included our experience with NPS cysts. METHODS: a systematic study of NPS cysts published in the English literature, between the periods of 1989-2008, was performed. The data were analyzed with respect to the type of cysts, mode of presentation, patient's demography, management modalities and complications. Data were quoted as median and range. Categorical data were analyzed using Chi(2) tests. P value ≤ 0.05 was considered as significant. All children, who presented to us with NPS cysts during the last 20 years, were reviewed and followed up till December 2009. RESULTS: after critical review of 249 abstracts, 25 studies (retrospective 13, cases reports 11 and prospective study 1) were finally included in this project. This included a total of 166 patients. The types of cysts were congenital (82%), traumatic (15%) and hamartomatous (3%). Male to female ratio was one to one. Median age of presentation was 11 years. Abdominal pain was the main complaint in 46% cases. Forty seven percent of the cysts were diagnosed incidentally. History of trauma was present in 11% cases. 1% cases were operated on due to the rupture of the cysts. Open procedures were performed in 60% cases [11% (total splenectomy), 29% (partial splenectomy) and 20% (cystectomy)]. Laparoscopic procedures were performed in the rest 40% cases [2% (total splenectomy), 4% (partial splenectomy) and 34% (cystectomy/partial decapsulation/unroofing)]. Median follow up period was 24 months (range 3-96 months). Recurrences were seen in only 2% of open procedures as Opposed to 41% in laparoscopic procedures (P<0.0001). Looking at the laparoscopic procedures individually, all the recurrences were seen in patients who had had cystectomy/partial decapsulation/unroofing. We treated 7 children (4 males and 3 females), who had a median age of 11 years (range 1-14 years); two of whom presented with large cysts having a diameter of 25 cm and 15 cm, respectively. They underwent open partial splenectomy without recurrence. The other five children had small cysts having a diameter of <5cm and they were asymptomatic. Presently, the children with small cysts are being followed up conservatively, with no concerns so far. CONCLUSIONS: congenital cysts are the most common NPS cysts in children. About half of the cysts are diagnosed incidentally. For bigger cysts, either open or laparoscopic partial splenectomy is the procedure of choice. Laparoscopic-cystectomy/partial decapsulation/unroofing procedures have unacceptably higher recurrence rates and hence should not be recommended. Smaller cysts (< 5 cm diameter) can be treated conservatively with regular ultrasound follow up, but if they become symptomatic or progress in size, surgical intervention is indicated.

Variations in heavy metal accumulation, growth and yield of rice plants grown at different sewage sludge amendment rates.

Use of sewage sludge in agriculture is an alternative disposal technique for this waste. The present field study was conducted to assess the suitability of sewage sludge amendment in soil for rice (Oryza sativa L. cv. Pusa sugandha 3) by evaluating the heavy metal accumulation, growth, biomass and yield responses of plants grown at 0, 3, 4.5, 6, 9, 12 kgm(-2) sewage sludge amendment (SSA) rate. Sewage sludge amendment modified the physico-chemical properties of soil, thus increasing the availability of heavy metals in soil and consequently with higher accumulation in plant parts. Root length decreased, whereas shoot length, number of leaves, leaf area and total biomass increased significantly when grown under various SSA rates. Yield of rice increased by 60%, 111%, 125%, 134% and 137% at 3, 4.5, 6, 9 and 12 kgm(-2) SSA, respectively, as compared to those grown in unamended soil. Sewage sludge amendment rates above 4.5 kgm(-2) though increased the yield of rice, but caused risk of food chain contamination as concentrations of Ni and Cd in rice grains were found to be above the Indian safe limits (1.5 mgkg(-1)) of human consumption above 4.5 kgm(-2) SSA and of Pb (2.5 mgkg(-1)) above 6 kgm(-2) SSA. Since aboveground parts of the rice also showed higher concentration than the permissible levels of Ni, Cd and Pb at 4.5 kgm(-2) SSA rate, it cannot be used as fodder. The rice husk may be used as bioresource for energy production. Efforts should be made to treat the effluents from small scale industries before discharge into the sewerage system.

Screening three cultivars of Vigna mungo L. against ozone by application of ethylenediurea (EDU).

Three Indian black gram cultivars (Vigna mungo L. cv. Barkha, Shekhar and TU-94-2) were grown at a tropical suburban site in Varanasi, India to evaluate the varietal differences in response to ambient O(3) under field conditions using ethylenediurea (EDU). EDU (400 ppm) was given as soil drench at 10-day intervals during the growth period of the cultivars. O(3) monitoring data clearly showed high concentrations with a mean value ranging between 41.3 and 59.9 ppb. EDU treatment caused significant increases in various growth parameters and total biomass accumulation in Barkha and Shekhar. EDU caused retention of more biomass in leaves during vegetative period and translocated more photosynthates towards reproductive parts, which resulted into yield enhancement. Weight of seeds plant(-1) was higher by 36.4% and 35.6% in Barkha and Shekhar, respectively, treated with EDU compared to non-EDU-treated plants. However, TU-94-2 did not exhibit any significant difference in weight of seeds plant(-1). Starch, total sugar, amino acids and K contents increased in seeds of EDU-treated plants leading to improvement in quality response index (QRI) of seeds. EDU helped in identifying the cultivar susceptibility to O(3) stress and therefore is very useful as a monitoring tool to assess the impact of ambient O(3) on plants under natural field conditions particularly in areas experiencing moderate concentrations of O(3).

Leprosy beyond MDT: study of follow-up of 100 released from treatment cases.

Appearance of new skin and/or nerve lesions during or after fixed duration of multidrug therapy (MDT), in leprosy, is not uncommon. It could be a lesion due to leprosy reaction or relapse. Differentiation is easy in classical reactions both clinically and histopathologically. But, difficult in other situations especially when the relapse cases present with features of reaction at the onset. A study was done to find the reasons for released from treatment (RFT) cases to come to clinic and to follow in terms of clinical and neurological activity, leprosy reactions and deformity progression. Out of them, 14 cases and 86 cases had received paucibacillary (PB) and multibacillary (MB) multidrug therapy respectively. Skin lesions either old or new were noticed in 74% cases which might be due to inactivity or activity were noticed in 74% cases which might be due to inactivity or activity in forms of relapse and reaction. Relapse was seen in 26 cases. Out of these, 10 and 16 cases were previously diagnosed as PB and MB cases respectively. PB cases relapsed into MB cases while MB cases relapsed into MB cases. 46 cases presented with either type 1 or type 2 reaction. After declared as RFT, parasthesia in 34 cases, weakness in 18 cases, paresis and paralytic deformity in 6 cases were seen. So, all the RFT cases need regular follow-up, IEC and physiotherapy to prevent deformity and to diagnose relapse and reactions at the earliest.

Biochemical and physiological responses of rice (Oryza sativa L.) grown on different sewage sludge amendments rates.

Using sewage sludge, a biological residue from sewage treatment processes, in agriculture is an alternative disposal technique of waste. To study the biochemical and physiological responses of Rice (Oryza sativa L.) grown on different sewage sludge amendments (SSA) rates a field experiment was conducted by mixing sewage sludge at 0, 3, 4.5, 6, 9, 12 kg m(-2) rate to the agricultural soil. Rate of photosynthesis and stomatal conductance increased in plants grown at different SSA rate. Chlorophyll and protein contents also increased due to different SSA rates. Lipid peroxidation, ascorbic acid, peroxidase activity and proline content increased, however, thiol and phenol content decreased in plants grown at different SSA rates. The study concludes that for rice plant sewage sludge amendment in soil may be a good option as plant has adequate heavy metal tolerance mechanism showed by increased rate of photosynthesis and chlorophyll content and various antioxidant levels.

Physiological, biochemical and growth responses of lady's finger (Abelmoschus esculentus L.) plants as affected by Cd contaminated soil.

Cadmium contamination of the soil is a common cause of environmental concern in the suburban areas of developing cities in India The present research paper describes the changes in physiological, biochemical, growth and yield characteristics, and bioaccumulation potential of lady's finger (Abelmoschus esculentus L.), an important vegetable crop at different levels of Cd in the soil. Cadmium accumulation was maximum in roots followed by stems, leaves and fruits at 100 mg Cd kg(-1) in the soil. Cd accumulation in lady's finger negatively affected the physiological and biochemical characteristics, growth and yield. The magnitude of negative effect enhanced with increasing Cd concentration. The study suggests that due to higher potential of bioaccumulation of Cd in lady's finger and consequent reductions in growth and yield, this plant may not be a suitable option for cultivation in Cd contaminated soil.

Responses of two cultivars of Trifolium repens L. to ethylene diurea in relation to ambient ozone.

Three ethylene diurea (EDU) concentrations (0, 150 and 300 mg/L) were used to evaluate the negative impact of ozone (O3) on two cultivars of Trifolium repens L. cv. Vardan and Bundel grown under natural field conditions in a suburban area of Varanasi, India. Mean O3 concentrations varied from 30.3 to 46.6 microg/L during the experimental period. Higher photosynthetic pigments and ascorbic acid concentrations were noticed in both EDU-treated cultivars over non-EDU-treated ones, but a reverse trend was found for lipid peroxidation. Growth parameters and biomass also showed increments under EDU treatment of both cultivars. The ratio of variable fluorescence to maximum fluorescence increased significantly in Vardan but not in Bundel upon EDU treatment. Results revealed that EDU concentration of 300 mg/L was more effective to combat the oxidative stress as well as protecting plants from O3 injury symptoms. The test cultivar Vardan is relatively sensitive to O3, thereby can be used as a bioindicator of O3 pollution in areas having higher O3 concentrations. Results also indicated that Bundel has more efficient antioxidant defense system than Vardan and hence was more tolerant to O3 stress.