”TAM”ing of the shrew-challenges in the diagnosis of Neonatal leukemia with Down's syndrome -A case report with literature review

The latest WHO (2017) classification describes the hematological abnormalities of Down's syndrome as a separate entity under 'Myeloid proliferations associated with Down's syndrome'. It includes Transient Abnormal Myelopoiesis and Myeloid leukemia of Down's syndrome. Here we report a case of a 3 days old neonate with Down's syndrome, presenting with a leukemic blood picture. The baby had icterus, fever and hepatosplenomagaly. Peripheral blood showed megakaryoblasts and giant platelets. A diagnosis of transient abnormal myelopoiesis was made by confirming with karyotyping and immunophenotyping. We attempt to address all the diagnostic challenges faced by a clinician and pathologist same, upon encountering such a case,by following an algorithmic approach. The mandatory need for follow up and cytogenetic studies in identifying high risk cases that will become myeloid leukemia of Down's syndrome are stressed. Our case also throws light upon the significance of identification of GATA1 mutation in diagnosing and prognostication of such cases.

Diversity of Drimia indica (Roxb.) Jessop and its relationship to Drimia nagarjunae using phenotypic traits and molecular markers.

In the present study, we explored the diversity of different accessions of Drimia indica and its relation to D. nagarjunae using phenotypic traits and molecular markers. Twenty populations of D. indica, from different parts of India, were compared with D. nagarjunae, an endangered medicinal plant collected from Andhra Pradesh, India. Two species showed appreciable phenotypic diversity in number of leaves, leaf indices, bulb circumference, bulb length and length of roots. The principal component analysis (PCA) performed on above 5 quantitative characters to determine relationship among populations, has distinguished D. nagarjunae from D. indica phenotypically. Genetic diversity was analysed using RAPD and ISSR primers which produced reproducible bands in 8 RAPD and 3 ISSR primers. A total of 89 amplicons were observed, of which 69 (77.53 %) were polymorphic. Cluster diagram and phylogenetic linkage showed that D. nagarjunae formed a separate cluster, showing no similarity with any of the populations of D. indica. The molecular marker data correlated with PCA of phenotypic traits. Current investigations have demonstrated that the statistical approach for phenotypic characters and molecular markers analysis can be applied to study diversity in Drimia species.

Subcutaneous intralesional Ksharodaka injection: A novel treatment for the management of Warts: A case series.

Warts are generally managed using cryosurgery, keratolytic ointments, curettage and electrodessication. Warts, vis‑a‑vis Charmakila, in Ayurvedic classical texts are classified into different types depending on the dominance of dosha. Ayurveda prescribes oral medications, topical use of Kshara (alkaline ash of herbs), Agni (thermal cautery) and Shastrakarma (surgery) for removal of Charmakila. Use of topical Kshara in the form of powder, aqueous solution i.e. Ksharodaka and Ksharasutra (thread smeared with Kshara) for warts has been reported. However, these methods necessitate multiple sittings and takea longer duration for removal of the warts. Herewith, we report a case series of different types of warts treated with intralesional infiltration of Apamarga Ksharodaka (AK), i.e. aqueous solution of Apamarga (Achyranthes aspera) Kshara. We observed that all these warts took a minimum of 2–6 days to shed off, leaving minor scars. There were no adverse reactions reported in any of these cases.

Basti: Does the equipment and method of administration matter.

Basti is one of the fi ve procedures of panchakarma in Ayurveda. Classically, it is advocated in the diseases of vata. It is mainly of two types viz. asthapana and anuvasana. According to the classical texts basti administration is done with the help of animal bladder (bastiputaka) and specially prepared metal/wooden nozzle/catheter (bastinetra), the whole assembly is called as bastiyantra. Nowadays, except in some of the Vaidya traditions in Kerala, basti administration is often done using enema-can or douche-set. In the aforesaid classical procedure active pressure is expected to be given on the bastiputaka whereas, in conventionally used enema-can only passive or gravitational force plays a role. This is important in the context of ‘basti danakala or pidanakala i.e. time for basti administration’.

Cerebral aneurysms in atrial myxoma: a delayed, rare manifestation.

Atrial myxomas are the most common primary tumors of the heart. Neurologic involvement usually occurs as a stroke with ischemic episodes. Following excision of cardiac myxomas, delayed neurologic events owing to aneurysms are rare and have not been reported from India. We report an operated case of left atrial myxoma. The patient initially presented with a stroke and 6 months after the surgery, developed multiple intracerebral hemorrhages due to the rupture of fusiform cerebral aneurysms, without recurrence of the cardiac tumor.

Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.

Degradation of 3-hydroxybenzoic acid by a Bacillus species.

A Bacillus sp. isolated by 4-hydroxybenzoate enrichment culture technique is capable of utilizing 3-hydroxybenzoate as the sole source of carbon and energy. The organism degraded 3-hydroxybenzoic acid through the intermediate formation of protocatechuic acid. 3-Hydroxybenzoate 4-hydroxylase, protocatechuate 3,4-dioxygenase and protocatechuate 4,5-dioxygenase activities were shown in cell-free extracts. The formed protocatechuate is metabolized through both the ortho and meta cleavage pathway.

Degradation of naphthalene by a Pseudomonas strain NGK1.

A Pseudomonas strain NGK1 capable of degrading naphthalene as a sole carbon source was isolated from biological waste treatment effluent. After 48 hr of incubation in 0.1% naphthalene mineral salts medium, the bacterial culture showed irregular clumped cells and salicylic acid (68 micrograms per ml) in the medium (pH 3.8). The strain degraded naphthalene through salicylate and catechol as was evidenced by metabolite characterization, oxygen uptake and enzymatic studies.

Malignant melanoma of choroid.

Four cases of malignant melanoma of the choroid are reported due to rarity of the condition in India. One of the cases presented with Naevus of Ota. All the cases had typical clinical and investigative features. All cases were enucleated. Histopathologically three of them were of mixed type and one was of the epithelioid type. Two of the cases were seen in patients below 40 years of age.