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D Follman

Publications and source records attributed to D Follman.

8 recordsLinked to original sources

Clinical and laboratory evidence for a trilineage haematopoietic defect in patients with refractory Diamond-Blackfan anaemia.

Diamond-Blackfan anaemia (DBA) is a constitutional pure red cell aplasia presenting in early childhood. In some patients, neutropenia and/or thrombocytopenia have also been observed during the course of the disease. We have followed 28 patients with steroid-refractory DBA for up to 13 years with serial peripheral blood counts and bone marrow (BM) aspirates and biopsies. In 21/28 (75%) patients, moderate to severe generalized BM hypoplasia developed, with overall cellularities ranging from 0% to 30%. Marrow hypoplasia correlated with the development of neutropenia (9/21; 43%) and/or thrombocytopenia (6/21; 29%) in many patients. No patient had either cytogenetic abnormalities or progressed to acute leukaemia, although one 13-year-old developed marked marrow fibrosis and trilineage dysplasia. We used the in vitro long-term culture-initiating cell (LTC-IC) assay to quantify multilineage, primitive haematopoietic progenitors in a representative subset of these patients. LTC-IC assays showed equivalent frequencies of cobblestone area-forming cells (CAFCs) with a mean of 5.42/10(5) cells +/- 1.9 SD and 6.13/10(5) cells +/- 2.6 SD in nine patients and six normal controls respectively. The average clonogenic cell output per LTC-IC, however, was significantly lower in DBA patients (mean 2.16 +/- 1.2 SD vs. 7. 36 +/- 2.7 SD in normal controls, P = 0.0008). Our results suggest that the underlying defect in patients with severe refractory DBA may not be limited to the erythroid lineage, as was evidenced by the development of pancytopenia, bone marrow hypoplasia and reduced clonogenic cell output in LTC-IC assays.

Adolescent↗

Ischemic heart disease in patients with end-stage renal disease.

Prevention and treatment of cardiovascular disease and, in particular, ischemic heart disease (IHD) in an increasingly elderly and diabetic population of patients with end-stage renal disease (ESRD) pose a challenge to all members of the renal failure treatment program. The patient described herein presents such a challenge, illustrating risk factors for IHD, the dilemma of performing surgery given his age and comorbid conditions, and the impact of IHD on his quality of life and capacity to function independently. The coordinated efforts of the nephrologist, cardiologist, cardiac surgeon, rehabilitation specialist, nutritionist, primary nurse, and social worker all contributed to a successful intervention.

Aged↗

Prevention and Treatment of Hypertension Study (PATHS). Rationale and design.

Alcohol consumption has been recognized as an important correlate of blood pressure in many epidemiologic studies, but few interventional studies have been conducted to examine the effect of a reduction in alcohol intake on blood pressure. Because these studies have usually included few subjects and been of short duration, the National Heart, Lung, and Blood Institute (NHLBI), the National Institute on Alcohol Abuse and Alcoholism (NIAAA), and the Veterans Affairs (VA) Cooperative Studies Program have initiated a randomized, controlled, multicenter trial to determine whether blood pressure and left ventricular mass are lowered over 6 months of alcohol moderation in non-dependent moderate to heavy drinkers (three or more drinks per day average but not alcohol dependent) with above-average normal (80 to 89 mm Hg) and mildly hypertensive (90 to 99 mm Hg) levels of diastolic blood pressure, and whether a reduction in alcohol intake can be maintained for 2 years. Eligible veterans are randomized to either an alcohol reduction intervention or a control observation group at seven clinical sites. The projected sample size is 580 participants. Alcohol intake is assessed by self-report using a retrospective diary (Chronological Drinking Record) and by various biochemical markers, including apolipoproteins, HDL cholesterol (and subfractions), and carbohydrate deficient transferrin, analyzed at a central laboratory. The alcohol intervention technique is a cognitive-behavioral program, the intensive phase of which consists of six counseling sessions over 3 months. Echocardiograms are obtained at baseline and 6 months after randomization. This trial has important implications for both the prevention and treatment of hypertension.

Adult↗

Double-chambered left ventricle: submitral accessory valvular tissue causing inlet and outlet obstruction.

We report a patient with a large submitral ridge of muscular and fibrous tissue that divides the left ventricle into two distinct chambers causing inlet and outlet obstruction. Doppler echocardiography revealed obstruction to both filling and ejection. Echocardiography demonstrated that the obstruction was in series with the mitral apparatus. Surgery was done with resection of much of this ring of tissue. Subsequent studies revealed morphologic and hemodynamic improvement.

Adult↗