Unilateral left Pulmonary Artery Agenesis with Pulmonary Hypertension: Unusual First Presentation.

Introduction: Unilateral absence of pulmonary artery is arare congenital disorder. We present here the case of Unilateralleft pulmonary artery agenesis with pulmonary hypertension.Case Report: A 33-year young male patient presented toour tertiary care hospital in the out-patient department withcomplaints of shortness of breath on exertion for 7 months andbilateral feet swelling for 6 months. Routine investigationswere within normal limits except 2 dimensional ECHOshowed right atrium and right ventricle dilatation with severepulmonary artery hypertension and 60% left ventricularejection fraction. Patients with isolated right pulmonary arteryagenesis commonly survive into adulthood with minimal orno symptoms, which makes their identification challenging.But our patient had left pulmonary artery agenesis whichhas significant association of life threatening cardiovascularmalformations with left-sided UAPA and surgical repair isoften required during the first year of life. None of which ispresent in our patient which is unsusual.Conclusion: Clinicians should be aware that recurrentrespiratory infections may be presenting feature ofUAPA. Initial investigation is usually a chest radiograph.Echocardiography is required for evaluation of possiblepulmonary hypertension. Confirmation of the diagnosis andanatomic details can be discerned by CT scanning and MRI.Angiography is reserved for patients requiring embolizationor revascularization surgery.Present case demonstrates a rare presentation of UAPA asright heart failure in first visit.

Hyperhomocystetenemia, MTHFR Mutation Presenting as Unprovoked Pulmonary Embolism.

Introduction: For MTHFR as with homocysteine testing,no official guidelines exist as to who should be tested.Homozygosity for the MTHFR C677T mutation has beenassociated with an increase in blood clotting together withplasma homocysteine increase and DVT occurrence risk.Case report: A 28 year young male patient presented withcomplaints of sudden onset breathlessness for 5 days. Theepisodes of breathlessness were associated with diffuseanterior chest pain. There was no history of leg pain, cough,sputum, hemoptysis, fever. No history of prior hospitalization,trauma, surgery and immobilization could be elicited from thepatient. He was a non smoker with no other comorbidities.On presentation his pulse rate was 120 per minute, respiratoryrate was 22 per minute, blood pressure 146/92 mm Hg,temperature 98.8 ° F, SpO2 of 94% at room air. His generalphysical examination was unremarkable.Conclusion: Although it has been observed that elevatedhomocysteine levels are a common finding in patientswith cardiovascular disease and thrombosis, its role in itspathogenesis is still under evaluation. Homozygosity forthe MTHFR C677T mutation has been associated withincreased homocysteine levels. Testing for this mutation is animportant parameter in thrombophilia workup of patients withunprovoked VTE.

Basaloid Squamous Cell Carcinoma of Oesophagus: Definitely Rare but does it Differs Significantly from the Conventional Squamous Cell Carcinoma.

Introduction: Basaloid squamous cell carcinoma (BSCC)is a rare, poorly differentiated variant of squamous cellcarcinoma (SCC). The diagnosis is made only after exclusionof conventional squamous cell carcinoma, adenoid cysticcarcinoma and small cell carcinoma. Our aim is to presenttwo cases of BSCCs identified in oesophagus with detailedclinicopathological, histological and immunohistochemicalfindings for better understanding of this rare entity which haslimited literature available to avoid misdiagnosis.Case Report: A detailed study of clinical and pathologicparameters in two cases of BSCC reported in our departmentwho had underwent potentially curative surgical resectionafter a preliminary post biopsy diagnosis was made.Microscopically, both the cases showed a quite similarpicture; they were composed of relatively small tumour cells,arranged in solid lobules with abundant comedo- necrosis.However, there were some minor variations as one of themwas accompanied by large areas of typical conventional SCC,whereas the other one showed presence of bizarre cells. Onimmunohistochemical analysis, the tumour cells showedstrong positivity for pan-CK with a high Ki67 index of 80-85%.Conclusion: BSCC have a poorer prognosis than conventionaloesophageal squamous cell carcinoma, but no definitivespecific treatment protocol has been established till date.Still these cases were considered worthy of discussion due tothe distinctness of this entity especially considering the siteoesophagus where it is regarded to be quite rare, in addition toits aggressiveness and poor outcome.

Effects of Gabapentin on Postoperative Pain after Laparoscopic Cholecystectomy

Introduction: Postoperative pain prevention and treatmentcontinues to be a major challenge in postoperative care.Gabapentin has recently become a part of a wide array ofpostoperative multimodal analgesic regimens. Present studyevaluated the efficacy of oral gabapentin in relief of acutepost-operative pain in laparoscopic cholecystectomy and alsocompared the efficacy of oral Gabapentin with that of oralTramadol.Material and Methods: Sixty ASA I and II physicalstatus patients of both sexes between 20-60 years of agescheduled for elective laparoscopic cholecystectomy wereenrolled into this prospective, double blinded, randomizedsequential allocation study. Any patient who was unwillingand uncooperative, ASA III and IV physical status, patientshaving body weight exceeding 20% of ideal body weight,known hypersensitive to any drug, drug or alcohol abuse,pregnant patient, uncontrolled concomitant medical diseases,history of chronic pain conditions, impaired kidney or liverfunction, laparoscopic cholecystectomy converted to opencholecystectomy, in whom some kind of analgesics wereadministered within 48 hrs of planned surgery were excludedfrom the study.Result: Sixty patients (30 males) were enrolled in the studywith mean ages of all three groups range from 37.40±9.18to 41.70±6.84. However the mean age, weight and sexdistribution among different groups were statisticallyinsignificant (P>0.05). Mean heart rate in various groups atdifferent intervals were insignificant (p>0.05) in the intraoperative period. Among group I, II and III in the postoperativeperiod, changes in mean heart rate was statistically significant(p<0.05). The changes in mean SBP and DBP werestatistically insignificant (p>0.05) intraoperatively in the threegroups.Conclusion: Premedication with oral 300 mg gabapentinprovides better pain relief in the postoperative period ascompare to oral 100 mg tramadol and placebo group withminimal side effects.

Candin Test in Evaluation of Cellular Immunity in Chronic Kidney Disease Patients - A Cross Sectional Observational Study

Introduction: Chronic kidney disease (CKD) patients are moresusceptible to infection due to impaired immune competency.Age, nutritional deficiencies, uremic toxins, dialysis,metabolism of parathyroid hormones and immunosuppressivemedications contribute to immune dysregulation. Aims ofthe present study were to find out the use of Candin Test toearly reorganization of immunocompromised state of cellularimmunity in patients of CKD and to find out correlationbetween Candin Test results and various factors alteringimmune system in CKD patients.Material and methods: The Cross-sectional observationalstudy was conducted on eighty adult patients qualifying thediagnostic criteria as per KDIGO guideline. Another ageand sex matched eighty healthy volunteers were selectedas control group. Patients having diabetes, HIV positive,malignancy, pregnancy, who are on steroids or any otherimmunosuppressive therapy, and those belonging to extremesof ages i.e.<18 years and >65 years were not included in thestudy. A detailed history, clinical examination and relevantinvestigations (Serum urea, creatinine, FBS, HIV, iPTH andUSG abdomen) were done in each subject. Each subject alsounderwent Candin Test to assess the level of cellular immunity.Result: Out of 80 cases, 35% patients showed positiveinduration while control group (n=80) demonstrated 58.8%indurations that revealed significantly more induration positivein controls (p=0.0024). In stage 3 CKD patients, 55.5% casesshowed positive induration, however positive indurationdecreases 52% and 24% in stage 4 and stage 5 respectively.Induration was significantly more positive in stage 3 and stage4 in comparison to stage 5 (p=0.007). Longer duration of CKDshowed lesser number of positive induration response butdifference was statistically non-significant (p=0.0521). Therewas significantly more induration response in no hemodialysisgroup (p=0.032) in comparison to hemodialysis group. Nosignificant difference was observed between induration andage. Mean eGFR of the patients with positive induration was20.14±13.083 and those without induration was 12.87±7.968with p=0.003, which is significant i.e. cases with positiveinduration have higher eGFR values. Mean Serum iPTH is215.50±119.279 in patients with positive induration and312.88±286.601 in patients with no induration which is notsignificant (p=0.091).Conclusion: Our study emphasises that Candin Test maybe useful to predict anergy in patients of CKD. As the CKDpatients approaches towards ESRD, cellular immunityalso decreases. It has also been observed that Candin Testresponse was diminished in patients on maintenance HD,which explains that as the frequency of HD increases patient'simmunity decreases.