Klippel Trenaunay Syndrome: A Case Report.

Klippel Trenaunay syndrome refers to a rare congenital anomaly which is characterized by capillary malformation, venous malformation and sometimes lymphatic malformation associated with overgrowth of a limb, with soft tissue hypertrophy and/or bony hypertrophy. The anomaly, if present, is present at birth and usually involves the lower limbs as well as portion of trunk, face, uppper limb or head. Our reporting case is a preterm male neonate having port wine stain, varicose veins and excessive growth of soft tissue of left lower limb clinically consistent with Klippel Trenaunay Syndrome.

Incomplete Pentalogy of Cantrell--A Case Report.

Cantrell's Pentalogy is a rare congenital malformation consists of supraumbilical abdominal wall defect, defect in the lower part of sternum, agenesis of anterior portion of diaphragm, an absence of the diaphragmatic part of the pericardium and intracardiac malformation. This case report presents a female neonate, who was born at 40 weeks of gestation weighing 2400 gm and was admitted 4 hours after delivery with the complaints of something coming out from chest. On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized with cardiac pulsation. On investigation chest X-ray lateral view showed absence of lower part of sternum, echocardiography findings were different in different institutes. Echocardiography findings at Mymensingh medical college hospital (MMCH) were large VSD (Ventriculo septal defect), ASD (Atrial septal defect) and rotated heart. On colour Doppler ultrasonogram at MMCH showed there were VSD, ASD, and a small epigastric swelling which was vascular and appears to be attached to the apex of the heart. On the other hand echocardiography findings of LAB AID hospital in Dhaka were Dextrocardia, complete AV (atrio ventricular) canal defect, almost common atrium, almost single ventricle, common AV valve, double outlet right ventricle (DORV), Cortriatriatum, mild A-V valve regurgitation and severe pulmonary hypertension (PAH). Echocardiography was also done at national heart foundation hospital in Dhaka. Findings were situs solitus, mesocardia to dextrocardia, DORV, large VSD, mild mitral inflow, mild TR (triuspid regurgitation), good LV (left ventricle) and RV (right ventricle) systolic function. Cardiologists at Dhaka in Bangladesh were suggested for surgery. Then the patient was consulted at Naryan Institute of Cardiac Science, Chennai in India. Here echocardiography findings were dextroversion/dextrocardia, DILV (Double inlet left ventricle), large inlet VSD with bidirectional shunt, mild TR, severe PAH with good ventricular function. Cardiologists in India were given comment about this patient. This patient was highly risky for surgery. They advised medical treatment and requested to review after one year. By taking medical treatment patient condition is well except failure to thrive and cyanosis develops during feeding and crying according to the statement of guardian of the patient. This case has 3 criterias among the five criteria of Cantrell's Pentalogy. So, it is incomplete Pentalogy of Cantrell.

Low-, medium- and high-dose steroids with or without aminocaproic acid in adult hematopoietic SCT patients with diffuse alveolar hemorrhage.

Diffuse alveolar hemorrhage (DAH) is a poorly understood complication of transplantation carrying a high mortality. Patients commonly deteriorate and require intensive care unit (ICU) admission. Treatment with high-dose steroids and aminocaproic acid (ACA) has been suggested. The current study examined 119 critically ill adult hematopoietic transplant patients treated for DAH. Patients were subdivided into low-, medium- and high-dose steroid groups with or without ACA. All groups had similar baseline characteristics and severity of illness scores. Primary objectives were 30, 60, 100 day, ICU and hospital mortality. Overall mortality (n=119) on day 100 was high at 85%. In the steroids and ACA cohort (n=82), there were no significant differences in 30, 60, 100, day, ICU and hospital mortality between the dosing groups. In the steroids only cohort (n=37), the low-dose steroid group had a lower ICU and hospital mortality (P=0.02). Adjunctive treatment with ACA did not produce differences in outcomes. In the multivariate analysis, medium- and high-dose steroids were associated with a higher ICU mortality (P=0.01) as compared with the low-dose group. Our data suggest that treatment strategies may need to be reanalyzed to avoid potentially unnecessary and potentially harmful therapies.

Value of red cell distribution width (RDW) and RBC indices in the detection of iron deficiency anemia.

Iron deficiency anemia is the common problem during pregnancy. Detection of iron deficiency early during pregnancy can reduce maternal and child mortality and morbidity. Red cell distribution width is a new routine parameter in fully automated hematology analyzer that can give the idea of early iron deficiency before other test. It gives the idea of red cell size variation which is the earliest morphologic changes in iron deficiency anemia. In prelatent and latent stage of iron deficiency MCV are normal. Whereas in latent stage Red Cell Distribution Width (RDW) would be expected to increase because of a microcytic population of cells appears in the blood. This study evaluates the role of red cell distribution width and RBC indices in determining iron deficiency early and provide reliable and useful technique. In this study 190 pregnant women were included. RDW, MCV, MCH, MCHC and iron profile were done. RDW compared with MCV, MCH and MCHC in various stages of iron deficiency. In latent stage of iron deficiency higher RDW was found significant than MCV, MCH, MCHC (p<0.05). In this study RDW had sensitivity 82.3% and specificity 97.4%. Whereas MCV, MCH and MCHC had 29.2%, 68.1% and 15% sensitivity but specificity was 98.7%, 83.1% and 96.1% in the detection of iron deficiency. Iron deficiency anaemia without other complicating disease could be screened out early by increased RDW when RBC indices were normal.

Role of red cell distribution width (RDW) in the detection of iron deficiency anaemia in pregnancy within the first 20 weeks of gestation.

Iron deficiency anemia is common problem during pregnancy. Red cell size variation (anisocytosis) is the earliest morphologic changes in iron deficiency anemia. Red cell distribution width is a quantitative measure of red cell size variation and it can give the idea of early iron deficiency before other test to become positive. 190 pregnant women were included in this study. Red cell distribution width was compared between iron deficient & non-iron deficient pregnant women. Red cell distribution width also compared with Hb level, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration and peripheral blood film in prelatent iron deficiency, latent iron deficiency, mild and moderate iron deficiency anemia. Red cell distribution width had sensitivity 82.3% and specificity 97.4%. Whereas Hb level, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration and peripheral blood film all had 56.6%, 29.2%, 68.1%, 15% and 38.9% sensitivity but specificity was 90.9%, 98.7%, 83.1%, 96.1% and 98.7% in the detection of iron deficiency. Red cell distribution width appears to be a reliable and useful parameter for detection of iron deficiency during pregnancy.

Long-term results of surgical decompression of thoracic outlet compression syndrome.

BACKGROUND: Thoracic outlet compression syndrome is characterised by a variety of symptoms relating to compression of the neurovascular bundle. Though no one test is specific for the syndrome, relief of symptoms may be obtained following surgery in up to 99% of cases. PATIENTS AND METHODS: The notes of 118 patients operated on in 126 operations by a single surgeon using a supraclavicular approach were reviewed. Symptoms, pre-operative investigations, and complications were all documented. Outcome at 6 weeks, 6, 12 and 24 months follow-up was also recorded. In addition, 61 patients were contacted by telephone, in order to assess current level of symptoms. RESULTS: Symptoms were predominantly motor, sensory or vasomotor, and were present for a mean of 19.6 months prior to surgery. Complications were rare, but included a pneumothorax requiring a chest drain (n = 1) and infraclavicular anaesthesia (n = 13). The mean duration of hospital stay was 2.1 days. At 6 weeks follow up, 86.5% of patients reported either an improvement, or complete resolution of their symptoms. Sixty-one patients were contactable, a mean of 55 months following decompression. Of these, 44 (72.1%) were either improved or asymptomatic. CONCLUSION: Decompression for thoracic outlet compression syndrome through a supraclavicular approach encompassing first rib resection leads to good long-term results with few complications.