Rapp-Hodgkin ectodermal dysplasia

Rapp-Hodgkin ectodermal dysplasia is an autosomal dominant condition characterized by distinctive craniofacies, cleft lip or palate, dental hypoplasia with caries, dystrophic nails, and decreased to absent sweat glands and hair follicles. We report the third family to be described, and distinguish the condition from similar syndromes.     

THE BEHAVIOR OF RENAL BLOOD FLOW AFTER PARTIAL CONSTRICTION OF THE RENAL ARTERY

1. Studies have been made of the behavior of renal blood flow after partial constriction of the renal artery in twenty-four dogs. 2. When reduction in renal blood flow is produced by partial constriction of the renal artery, a readjustment of flow in the direction of normal occurs within a few minutes, subsequent constriction being again followed by a return of flow toward normal until the artery is markedly constricted. 3. Renal blood flow after marked constriction of the artery becomes extremely susceptible to the vasoconstrictive action of small doses of adrenalin, and flow may cease with larger doses for a considerable period of time. 4. Arterial hypertension of significant degree may follow partial constriction of one renal artery during brief experiments when adrenalin in addition has been administered. 5. Further evidence is presented in favor of the concept that the renal circulation enjoys a control independent of systemic arterial pressure.     

Arbeiten aus der I. Medizinischen klinik der Universität München C-Vitaminbedarf und C-Hypovitaminose

Ohne Zusammenfassung     

Review of intravenous immunoglobulin replacement therapy trials for primary humoral immunodeficiency patients

An available supply of intravenous immunoglobulin (IVIG) is essential for individuals with primary humoral immunodeficiency. A shortage in 1997 prompted the Food and Drug Administration (FDA) to revise guidelines for the licensure, production, and distribution of new IVIG products, including the standardization of United States clinical trials regarding endpoints for safety, efficacy, and pharmacokinetics. The following review is intended to present current information and results of clinical trials in patients with primary immunodeficiency treated with IVIG products currently licensed or awaiting licensure in the United States. The data presented are compiled from published clinical trials and prescribing information generated by manufacturers.     

The law medicine letter: Treatment Of Juveniles Without Parental Consent

The needs of the child—not the fault of the parents—are the basis for the law's intervention in the welfare of a child. In an emergency, the physician or surgeon has not only the right but also the legal duty to save the life of a child. Even if he cannot locate the parents to obtain their consent, he may act, especially when the patient is a mature juvenile.     

Determination of the renal concentration capacity following intravenous administration of dDAVP in healthy humans

The synthetic AVP analogue 1-desamino-8-d-arginine-vasopressin (dDAVP) is used for treatment of polyuric disorders. Lack of commercially available assays limits the usefulness of dDAVP as a diagnostic tool in the assessment of renal concentrating capacity. We aimed to develop a specific radioimmunoassay (RIA) for determination of plasma dDAVP (pdDAVP) in order to investigate the relationship between pdDAVP levels and urine osmolality (Uosm). Further, we aimed to determine the onset, duration, and maximum concentrating capacity following intravenous (i.v.) bolus dDAVP injection. The dDAVP assay was based on a well-established RIA for measurements of AVP. Fourteen healthy subjects (aged 15–18 years) participated. Blood and urine samples were collected prior to and after i.v. bolus of 0.03?µg/kg dDAVP. Diuresis and Uosm was measured for nine hours following dDAVP administration. PdDAVP and Uosm were analyzed.We established a specific RIA for the measurement of pdDAVP. All subjects reached maximal pdDAVP concentration (C_max) 30 minutes following infusion, and a rise in Uosm after 60 minutes. Maximal Uosm varied between subjects, with no direct correlation to the achieved pdDAVP levels. We found no significant intra-individual variation between two dDAVP infusions and the effect was reproducible in terms of C_max and maximal Uosm. We characterized the relationship between pdDAVP and Uosm after dDAVP bolus injection in healthy adolescents using our dDAVP assay. Maximal Uosm achieved correlated with the baseline Uosm levels and seemed unrelated to achieved pdDAVP levels. The urine concentrating response was maintained at least eight hours.