A 69-year-old woman with persistent iron deficiency anemia

In women, iron deficiency anemia—a result of chronic iron loss—is most common during the reproductive years because of physiologic demands such as menstrual blood losses and pregnancy. In other cases, iron deficiency anemia is generally attributed to occult gastrointestinal bleeding. Common causes of chronic gastrointestinal blood loss include erosive esophagitis, gastric and duodenal ulcers, vascular ectasias, colon adenomas, and cancers. Bleeding from the small intestine at sites beyond the duodenal bulb is uncommon. The lesions of the small intestine are responsible for approximately 4% of gastrointestinal bleeding [7]. In this report we describe a case of persistent iron deficiency anemia due to carcinoid tumor of the small intestine.     

Hepatitis B Virus Markers in Hematologic Patients: Relation to Transfusion Treatment and Hospitalization

Abstract. Screening tests for hepatitis B virus (HBV) markers were performed in 266 hematologic patients in order to evaluate the role of transfusion therapy in HBV infection and to identify other possible causes of the high rate of HBV markers positivity in onco-hematologic units. As control groups we tested 99 nonhematologic polytransfused patients, 66 nonhematologic, nontransfused inpatients with various diseases and 72 subjects randomly selected from the general population. Higher HBV markers prevalence was found in hematologic patients, nonhematologic polytransfused patients and nonhematologic, non-transfused inpatients than in the general population. HBV markers prevalence correlated with the length of hospitalization in all inpatients studied. Our data suggest that hospital admission is a major factor in HBV transmission in hematologic patients and in other inpatients studied. Blood transfusions represent a risk factor only when utilized as chronic treatment.     

Gender effect on onset,prevalence and surgical treatment of cataract in patients with Myotonic Dystrophy type 1

Myotonic Dystrophy type 1 (DM1) is the most common muscular dystrophy in adults, affecting 1:8000 individuals. It is a multi-systemic disorder involving muscle, heart, endocrine and respiratory apparatus and eye. The eye symptoms can include ptosis, external ophthalmoplegia, epiphora, and early onset cataracts. Cataracts occur at a much earlier age (usually between 30 and 40) than the general population, where females are usually affected more than men. We studied gender differences in cataract prevalence and treatment age in 243 DM1 patients (134 M; 109 F), aged 18 to 70 years, who were subsequently screened at routine follow-up. For each patient, information was collected on age, sex, CTG expansion, age of cataract onset, and age at cataract surgery, when available.Seventy-three patients, 30 females and 43 males, had cataracts, at a mean age of onset of 41.14 ± 12.64 in females, and 40.36 ± 10.03 in males. Sixty-nine of them underwent cataract surgery, males at an earlier age than females (42.8 ± 9.8 years versus 47.3 ± 12.6 years) and in 52.5% of cases before the age of 40, compared to 17.2% of females. The difference was statistically significant. The assumption that females in general and those with DM1 in particular develop cataracts more frequently and earlier than males is not confirmed, at least in this study. A possible explanation for these results could be related to non-advanced age, the protective role of estrogen and the lower prevalence of smoking in the study population.Key words: Myotonic Dystrophy type 1, Steinert disease, gender, cataract, cataract surgery, prevalence     

A preliminary randomized study of growth hormone administration in Becker and Duchenne muscular dystrophies.

AIM: Since growth hormone (GH) has proven beneficial in experimental heart failure, and the natural history of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) is frequently complicated by the development of dilated cardiomyopathy, we administered GH to six patients with DMD and 10 with BMD, with the evidence of cardiac involvement. METHODS AND RESULTS: Patients were randomized to receive for 3 months either placebo or recombinant human GH, in a double-blind fashion. In GH-treated patients, left ventricular (LV) mass increased by 16% in BMD and by 29% in DMD (both p<0.01), with a significant increase of relative wall thickness (+19%). Systemic blood pressure remained unchanged, while LV end-systolic stress fell significantly by 13% in BMD and by 33% in DMD, with a slight increase of systolic function indexes. No changes were observed related to cardiac arrhythmias and skeletal muscle function in the patient groups during the treatment period, nor any side effects were observed. Brain natriuretic peptide, interleukin-6, and tumor necrosis factor-alpha circulating levels were elevated at baseline. While brain natriuretic peptide decreased by 40%, cytokine levels did not exhibit significant variations during the treatment period. CONCLUSIONS: The 3-month GH therapy in patients with DMD and BMD induces a hypertrophic response associated with a significant reduction of brain natriuretic peptide plasma levels and a slight improvement of systolic function, no changes in skeletal muscle function, and no side effects.     

Evaluation of the cardiomyopathy in becker muscular dystrophy

To evaluate the features and the course of cardiomyopathy in Becker muscular dystrophy, 68 patients–identified by clinical assessment and by reduced dystrophin labeling and/or DNA analysis–were followed in the years 1976–1993, for periods ranging from 3 to 18 years (mean 8). Patients periodically underwent clinical, electrocardiographic, echocardiographic, nuclear, and radiological assessments. Preclinical cardiac involvement was found in 67.4% of patients under 16 years of age, decreasing to 30% in patients older than 40. Clinically evident cardiomyopathy was found in 15% of patients under 16 years of age, increasing to 73% in patients older than 40. A real, dilated cardiomyopathy is the most frequent type of myocardial involvement after the age of 20. Results show that the severity of cardiac involvement can be unrelated to the severity of skeletal muscle damage and confirm that cardiac dysfunction is a primary feature of Becker muscular dystrophy.© 1995 John Wiley &Sons, Inc.     

Prospective study of X-linked progressive muscular dystrophy in campania

Within the Campania region of southern Italy a prospective study on X-linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of death.     

Distal muscular dystrophy with autosomal recessive inheritance

Two sisters presented with distal weakness and their muscle biopsy was dystrophic. This distal muscular dystrophy has an autosomal recessive inheritance and its features are somewhat different from the more common autosomal dominant distal muscular dystrophy and include: (a) onset in early adult life: (b) involvement of distal leg muscles and especially peroneal muscles; (c) marked early elevation of serum creatine kinase (CK); (d) brief duration, small amplitude motor units and fibrillation on electromyography; and (e) histologic features of a dystrophic myopathy.     

Meetings with Parents After the Death of Their Child from Leukemia

There is a growing interest in the emotional status of parents and siblings after the death of a child with chronic disease. For the past 7 years physicians at our center have systematically contacted parents who lost a child because of leukemia within the first few months after the death. From this experience it appears that most parents needed to talk at least once with the physicians who took care of their child. As expected, some parents and siblings were found to have significant psychological problems and to need psychologic support. We suggest that the opportunity to talk with a physician of the attending staff should be provided routinely to parents shortly after the death of a child from leukemia.