A second reported malignancy in a patient with Morquio syndrome

Morquio syndrome is a rare lysosomal storage disease that affects multiple organ systems. However, it is rarely associated with malignancy. We present the case of a 30-year old man with Morquio syndrome associated with gastric adenocarcinoma. This case also demonstrates two other findings that have not been previously described in patients with Morquio syndrome - malrotation of brainstem and cerebellum, without clinical neurologic deficit, and persistence of fetal lobulation in the kidneys.

A Pilot Randomized Control Cross over Study Evaluating the Effectiveness and Safety of Mechanical Percussor Compared with Conventional Chest Physiotherapy in Adults with Productive Cough

Introduction: Conventional Chest Physiotherapy (CCPT) remains the mainstay of treatment for sputum mobilization in patients with productive cough such as bronchiectasis and “Chronic Obstructive Airway Disease” (COPD). However CCPT is time consuming requires the assistance of a physiotherapist and limits the independence of the patient. Mechanical percussors which are electrical devices used to provide percussion to the external chest wall might provide autonomy and greater compliance. We compared safety and efficacy of a mechanical chest percusser devised by Formedic Technology with conventional chest percussion. Methods: Twenty patients (mean age 64years) were randomly assigned to receive either CCPT or mechanical percussor on the first day and crossed over by “Latin square randomisation” to alternative treatment for 6 consecutive days and the amount of sputum expectorated was compared by dry and wet weight. Adverse events and willingness to use was assessed by a home diary and a questionnaire. Results: There were 13 males and 7 females, eight diagnosed as bronchiectasis and 12 COPD. The mean dry weight of sputum induced by CCPT (0.54g + 0.32) was significantly more compared with MP (0.40g + 0.11); p-value = 0.002. The mean wet weight of sputum with CCPT (10.71g + 8.70) was also significantly more compared with MP (5.99g + 4.5); p-value < 0.001. There were no significant difference in adverse events and majority of patients were willing to use the device by themselves. Conclusion: The mechanical percussor although produces less sputum is well tolerated and can be a useful adjunct to CCPT

Household Cost of Antenatal Care and Delivery Services in a Rural Community of Kaduna State; Northwestern Nigeria

Maternal mortality remains a leading cause of death among women of reproductive age. While Nigeria has only two percent of the global population; it contributes 10 to the global maternal mortality burden. Antenatal care (ANC) reduces the incidence of maternal mortality. However; financial capability affects access to antenatal care. Thus; the rural poor are at a higher risk of maternal mortality. Materials and Methods: A cross-sectional descriptive study involving 135 women (pregnant women and those who are 6 weeks postpartum). Structured interviewer-administered questionnaires were used for data collection. Data analysis was carried out using statistical package for social sciences software (version 17). Results: The average amount spent on booking and initial laboratory investigations were N77 (half a dollar) and N316 ($2); respectively. Per ANC visit; average amount spent on drugs and transportation were N229 ($1.5) and N139 ($0.9) respectively. For delivery; the average amount spent was N1500 ($9.6). On an average; ANC plus delivery cost about N3;365.00 ($22). There was a statistically significant association between husband's income and ANC attendance (X 2 = 2.451; df = 2; P = 0.048). Conclusion: Cost of Antenatal care and delivery services were not catastrophic but were a barrier to accessing antenatal care and facility-based delivery services in the study area. ANC attendance was associated with the income of household heads. Pro-poor policies and actions are needed to address this problem; as it will go a long way in reducing maternal mortality in this part of the country

Effect of pre-elclampsia on nucleated red blood cell in the pregnant women and fetal outcome

The Aim of this study was to evaluate the influence of pre-eclampsia on the cord and maternal nucleated red blood cell [NRBCs] count. Prospective study. This study was carried out on 50 pre-eclamptic pregnant women and 150 healthy pregnant women from those attending delivery on obstetrics, and Gynecology Department, Al-Azhar University Hospitals. Before the delivery 1ml of maternal venous blood and immediately after the delivery, 1ml of fetal side cord blood form 50 pre-eclamptic and 150 healthy pregnant women were collected separately in tubes containing 1.5 mg ethylene diamine tetra-acetic acid. Blood smears were prepared and stained using the Giemsa method. The number of NRBCs per 100 leukocytes in maternal and cord blood was counted and compared between the Two groups. Any correlation of the NRBCs count in maternal and umbilical cord blood was also evaluated. p-values <0.05 were considered significant. The mean [ +/- SD] NRBCs per 100 white blood cell [WBCs] level in cord blood of newborns in the pre-eclamptic group [18.2 +/- 31.8, range 0-142] was significantly greater than in the control group [6.2 +/- 8.1, range 0.36]. Low birth weight and intrauterine growth restriction showed a statistically significant relationship with abnormal NRBC count in pre-eclamptic patients. A significant correlation was found between the maternal and cord blood NRBCs count in the pre-eclamptic group. Fetal response to utero-placental insufficiency in pre-eclampsia leads to elevated NRBC in the cord blood. Particularly in the presence of low birth eight and intrauterine growth restriction. The positive correlation between maternal and cord blood NRBC counts in pre-eclamptic patients indicates that may be the hypoperfused placenta plays a role in the correlated alteration of the maternal and fetal NRBCs count

Evaluation of ultrasonographic examination prediction of spontaneous first trimester abortion

This study was carried out on 445 patients in the first trimester of pregnancy. They had one or more risk factors for abortion. History and clinical examination were done and abdominal ultrasonographic examination was carried out on day 43 to 56, 57 to 70 and 71 to 84 of the estimated gestational age [EGA]. At the end of the first trimester, the results showed that the total incidence of abortion was 17.5%. The three ultrasonic examinations showed that the incidence of abortion was very high when the shape of gestational sac [GS] was irregular, followed by the dumbbell shape and when the average diameter of GS or crown-rump length [CRL] was shorter or longer than that of equal to EGA. Measurement of CRL on day 71 to 84 was accurate in prediction of first trimester abortion. It is concluded that ultrasound examination is accurate in predicting first trimester abortion. If EHM is not seen by day 71 to 84 of EGA, missed abortion should be considered. For patient at risk of abortion, ultrasound examination is recommended to be carried out as early in pregnancy as possible and to be repeated-within two weeks for three times

Value of estimation of serum progesterone hCG, and antiphopholipid antibodies in prediction of spontaneous first trimester abortion

This study was carried out on 445 pregnant patients in the first trimester and had one or more risk factors for abortion. After taking history and clinical examination, estimation of serum progesterone, hCG and APA [ACA and APTT] were done on day 43 to 56 of the estimated gestational age. At the end of the first trimester, the results showed that total incidence of abortion was 17.5%. The following risk factors were associated with a high incidence of abortion: Elderly patients, abnormal weight, primigravidae, para, previous abortions, hypertension, autoimmune disorders and endocrine abnormalities. Patients with abnormally low serum progesterone or hCG levels had significantly a higher incidence of abortion than patients with normal levels. Antiphospholipid antibodies were positive in 12.4% of all patients, 25.5% of them had first trimester abortion. Aborted patients with positive APA had significantly a higher incidence of abortion and higher levels of IgG and IgM of ACA and prolonged APTT than aborted patients with negative APA, there was no significant difference between them regarding age, weight, MAP, progesterone and hCG level. Measurement of ACA was more accurate in the prediction of the first trimester abortion than APTT