Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease

Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases. ADPKD in this family is linked to PKD1. The family is composed of 26 members; 11 members with ADPKD, 4 members with CF, and 2 members with both diseases. Renal volumes measured by computerized tomography (CT), calculated creatinine clearances, and other clinical parameters in the family members with ADPKD and CF were compared with those in the family members with ADPKD alone, as well as to a large population of patients with ADPKD. The patients with CF and ADPKD, but not the CF heterozygote carriers with ADPKD, had less severe polycystic kidney and liver disease, as indicated by normal renal function; smaller renal volume, even when corrected for height and body surface area; and the absence of hypertension and liver cysts. These observations suggest that the coexistence of CF may reduce the severity of ADPKD.     

Risk factors for development of dehydration in children aged under five who have acute watery diarrhoea: a case-control study

Objective: To identify risk factors for development of dehydration in under five year olds with acute watery diarrhoea.Design: Hospital based unmatched case-control study.Setting: Diarrhoea Treatment Unit, Government Medical College Hospital, Nagpur, India.Participants: The study included 387 cases of diarrhoea having severe or moderate dehydration and 387 controls suffering from diarrhoea with mild or no dehydration.Risk factors: The study included infancy, female sex, religion, residing in urban slums or rural area, under nutrition, cessation of breast feeding during diarrhoeal episode, fluid intake decreased/stopped during diarrhoea, ORS not received, home available fluids (HAF) not received, both ORS and HAF not received, non-washing of hands by mother before preparation of food, after defaecation, after disposal of faeces, history of measles in the previous six months, frequency of stools >8/d, frequency of vomiting more than twice per day and temperature more than 99°F, as risk factors for development of dehydration.Statistical analysis: Univariate analysis included OR, 95% CI for OR and Chi-square test. Multivariate analysis was carried out by unconditional multiple logistic regression (MLR).Results: This study identified the significance of infancy, religion, severe undernutrition, non-washing of hands by mother before preparation of food, frequency of stool >8/d, frequency of vomiting >2/d, history of measles in previous six months, withdrawal of breast feeding during diarrhoea, withdrawal of fluids during diarrhoea and not giving ORS, HAF or both during diarrhoea, in the outcome of development of moderate or severe dehydration.Conclusions: Timely intervention in the preventable risk factors included in this study may prevent the development of moderate or severe dehydration in the children suffering form acute watery diarrhoea.     

A boy with McCune-Albright syndrome associated with GH secreting pituitary microadenoma. Clinical findings and response to treatment

The McCune-Albright Syndrome (MAS) is a sporadic rare disease first described in 1936 by McCune and separately by Albright. MAS is characterized by a triad of physical signs: café-au-lait spots, polyostotic fibrous dysplasia and autonomous endocrine hyperfunction. MAS is predominantly observed in girls and is rarely reported in males. We report the case of a 9-year old boy with gonadotropin independent precocious puberty, café-au-lait spots, polyostotic fibrous dysplasia and growth hormone hypersecretion.     

Multifetal pregnancy reduction in pregnancies with a monochorionic component

We conducted a retrospective, cross-sectional study to evaluate the efficacy, safety, and effect of first-trimester multifetal pregnancy reduction on procedure-related complications and obstetrical outcome in multiple pregnancies with a monochorionic component. Although procedure-related complications were relatively common, the obstetrical outcome was favorable in most cases when the monochorionic twin component was reduced.     

CT Diagnosis of an Abortion-Related Retroperitoneal Space Abscess

A female patient with a history of recent abortion was transferred in critically ill condition in our hospital. CT imaging on emergency basis depicted an abscess in the right retroperitoneal space that included the head of fetus, along with rupture of the right lateral wall of uterus. Spiral CT enabled prompt diagnosis and therapy of this rare abortion-related complication. KEYWORDS: CT-Spiral; Uterus; Complication; Rupture; Obstetrics.     

Pharmacological treatment of hyperthyroidism during lactation: review of the literature and novel data

Antithyroid drugs (ATD) are used as a first line treatment in thyrotoxicosis. Propylthiouracil (PTU), carbimazole (CMZ) and methimazole (MMI) are available. During absorption CMZ is bioactivated to MMI. Initially, mothers were not allowed to breastfeed during treatment with ATD. Newer studies minimized the risk for mother and infant. PTU should be preferred over MMI due to its lower milk concentration. Recent studies have shown severe hepatic dysfunction for both ATD, but especially for PTU, in hyperthyroid patients. Most of those cases were idiosyncratic, not-dose related and presented a latent period of occurrence. No biomarkers could predict hepatic damage. The American Thyroid Association (ATA) has recommended that PTU should not be prescribed as the first line agent in children and adolescents. Its use might be accepted in the first trimester of pregnancy for severe thyrotoxicosis or for patients with previous MMI adverse reactions. Considering the potential harmful effects of PTU, MMI should be used instead during lactation.     

Comparison of the effect of post-instruction multiple-choice and short-answer tests on delayed retention learning

Background

People forget much of what they learn, therefore students could benefit from learning strategies that yield long-lasting knowledge. Yet surprisingly, little is known about how longterm retention is most efficiently mastered. We studied the value of teacher made in class tests as learning aids and compared two types of teacher-made tests (multiple choice and short-answer tests) with a no test (control) to determine their value as aids to retention learning.

Method

The study was conducted on two separate batches of medical undergraduate students. This study compared two types of tests [multiple choice questions (MCQs) and short answer questions (SAQs)] with a no test (control) group. The investigation involved initial testing at the end of the lecture (post instruction), followed by an unannounced delayed retention test on the same material three weeks later. The unannounced delayed test comprising of MCQs and SAQs on the same material was given three weeks later to all the three groups.

Results

In batch I, the MCQ group had a higher mean delayed retention score of 10.97, followed by the SAQ group (8.42) and the control group (6.71). Analysis of variance (ANOVA) test and least significance difference (LSD) post hoc test revealed statistically significant difference between the means of the three groups. Similar results were obtained for batch II

Conclusion

Classroom testing has a positive effect on retention learning; both short-answer and multiple-choice tests being more effective than no test in promoting delayed retention learning, however, multiple-choice tests are better.