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Biomedical subjects

R Kaufmann

Publications and source records attributed to R Kaufmann.

At least 253 records · Page 14Linked to original sources
PubMed1988

[Disease course in 20 patients with an early diagnosis of phenylketonuria and hyperphenylalaninemia].

Twenty patients with PKU or hyperphenylalaninemia at ages 0.1 to 15.6 years (median age 6.2 years) were studied prospectively. In all children the condition had been diagnosed when they were neonates on the basis of an abnormal Guthrie test. To maintain plasma phenylalanine levels between 0.2-0.5 mM, dietary restriction of phenylalanine to 20-80 mg/kg daily (median 40 mg/kg) was necessary in 14 children. In children above 8 years, however, these plasma levels were frequently exceeded. In 6 children plasma phenylalanine levels were higher than normal diet. Height, weight and head circumference were within normal range in all patients at all ages. Determinations of DQ/IQ were done at 2, 4, 6 and 8 years of age and revealed values between 90-120 with a median of 102 in the 14 patients who were tested. Only 1 patient had IQ levels between 75-85 and attended special school. Nine other patients were in grade school performing averagely or above. This study confirms that early treatment and long-term follow-up of patients with PKU yield good results. Unsolved problems include duration of dietary treatment and the management of pregnancy in women with PKU.

Adolescent↗
PubMed1987

[Dermatosurgery in phlebologic ambulatory care].

In certain types of primary varicosis (residuals following crossectomy or stripping, incompetent perforating veins, reticular varicosis) dermatosurgical treatment under local anesthesia presents an alternative to sclerotherapy in outpatients. Practicable procedures include such techniques as dissection of incompetent perforating veins, phlebectomy, and exairesis of branches. The management of leg ulcers offers further possibilities of surgical approach in a phlebologic ambulatory. We discuss the methods, criteria of patient selection, and indications for these operations.

Ambulatory Surgical Procedures↗
PubMed1987

Mucosal proctectomy and ileoanal pull-through technique and functional results in 23 consecutive patients.

Mucosal proctectomy with ileoanal pull-through in the treatment of ulcerative colitis and familial polyposis provides a technique for the preservation of the anal sphincters and relatively normal mechanisms of continence. Five patients had straight ileoanal anastomosis while 18 had the construction of a J-pouch. A two-team approach was used for simultaneous abdominal and perineal procedures to facilitate a shortened operating time. A loop ileostomy was routinely used in the postoperative period and was closed an average of 4.5 months (range: 2-16 months) later without complication. Prolonged preoperative hospitalization was rarely necessary and outpatient steroid enema preparation was routinely used. There were no deaths. Nineteen patients with functioning pull-through procedures have been followed an average of 23 months (range: 3-42 months). Two other patients have not had ileostomy closure because of complications. The two remaining patients had intractable diarrhea and have since undergone conversion to a permanent ileostomy. The 19 patients are continent, having three to nine bowel movements each day. Nearly all wear a perineal sanitary pad because of rare, unpredictable leakage of small amounts of fluid, especially at night. Complications were significant in this group of patients. Intestinal obstruction was a frequent problem, occurring in 52 per cent of the entire series and necessitating reoperation in 22 per cent. Anal stricture was a problem in another five patients. A variety of other minor problems occurred and most were treated nonoperatively. In spite of moderate diarrhea and occasional leakage of stool, all patients with functioning pull-through procedures prefer their current status to life with an ileostomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenomatous Polyposis Coli↗
PubMed1987

Role of immediate versus delayed nephrectomy for failed renal transplants.

The role of timing of transplant nephrectomy after renal transplantation has not been well defined. This report compares the morbidity, mortality, and hospitalization costs of 37 patients undergoing transplant nephrectomy within 14 days after graft failure and return to dialysis, with 31 patients undergoing delayed transplant nephrectomy (less than 14 days after graft failure and return to dialysis). This analysis revealed that there were no significant (P greater than .05) differences in patient morbidity and mortality between these groups. There was, however, a substantial increase (P less than .05) in the cost of hospitalization in the delayed nephrectomy group. For this reason, we recommend early nephrectomy after renal transplant failure in order to minimize the cost of health care for these patients undergoing failed cadaver donor transplant nephrectomy.

Costs and Cost Analysis↗
PubMed1987

[Diffuse (presenile) hyperplasia of the sebaceous glands, a new entity? Successful treatment with 13-cis-retinoic acid].

We report the case of a young man with extreme sebaceous gland hyperplasia that occurred in a diffuse pattern of aggregated papular lesions involving the entire face, neck and upper chest, together with marked seborrhoea oleosa. Oral therapy with 13-cis-retinoic acid (isotretinoin) resulted in remarkable improvement within a few weeks. Parallels from our case are drawn to familial sebaceous hyperplasia, reported by Dypre et al. in 1980 [6], and to a case of a young man with severe sebaceous gland hyperplasia and facial seborrhoea, reported by de Villez et al. in 1982. We suggest that these types of seboglandular proliferative disorders be classified as diffuse (presenile) sebaceous gland hyperplasia in contrast to the well-defined senile circumscribed variant, and that they be regarded as a separate entity.

Adult↗
PubMed1986

[Bilateral nevus of Ota--a case report].

We report on a case of the bilateral type of nevus fuscocaeruleus ophthalmomaxillaris Ota, which is rarely seen in European countries. We refer to other possible disturbances associated with this disorder.

Adolescent↗
PubMed1986

[Dermatosurgical treatment possibilities in leg ulcers].

On he basis of our own investigational findings in patients suffering from leg ulcers during the past 3 years, we report on general aspects regarding preoperative treatment of ulcus cruris. In addition, we give an illustrated synopsis of current dermatosurgical treatment, especially referring to leg ulcers in venous disorders. Aside from local surgical procedures, we discuss the various techniques of skin grafting as well as surgical possibilities with regard to the treatment of incompetent perforator veins.

Humans↗
PubMed1986

Placental transfer of class G immunoglobulins treated with beta-propiolactone (beta-PL) for intravenous application--a case report.

Pregnant patients receive immunoglobulin preparations for the prevention of connatal viral infections of the newborn in the case of impending or manifest maternal infections. In the past, class G immunoglobulin preparations could only be given intramuscularly because of possible antigen independent complement activation by the Fc-part of the IgG molecule when administered intravenously. The I. M. application results in a delay in reaching maximum serum levels, and the levels are much lower compared to I. V. application. Today, I. V. tolerable IgG preparations exist because methods have been developed either to remove or to modify the IgG Fc-part. As the Fc-part is necessary for the placental transport of antibodies, the above mentioned manipulation can drastically impair the transport. In this study, an anti-HBs seronegative pregnant patient received an anti-HBs IgG preparation (greater than or equal to 500 IU anti-HBs) intravenously. These IgG molecules are rendered I. V. tolerable by modification of the Fc-part with beta-propiolactone (beta-PL). At delivery, the newborn's serum anti-HBs concentration was 13 mIU per ml, the mother's 35 mIU per ml. This result clearly demonstrates that IgG molecules which have been modified by treatment with beta-PL are transported from the circulation of the mother across the placenta to the fetus. The role of intravenously tolerable hyperimmunoglobulins with special respect to beta-PL treated preparations for the prevention of connatal infections is discussed.

Adult↗
PubMed1985

Recent advances in laser microprobe mass analysis (LAMMA) of inner ear tissue.

Maintenance of ionic gradients within the various fluids compartments of the inner ear requires transport active cellular systems at different locations. LAMMA analysis is ideally suited for detection of ions in microquantity on cellular levels overcoming many technical difficulties. The present paper summarizes the results of microprobe analysis obtained with laser induced mass spectrometry (LAMMA) supplemented by X-ray microprobe analysis of epithelial cell layers adjacent to the endolymphatic space in the cochlear duct, in the vestibular organ and in the endolymphatic sac. The possible role of inner ear as well as ocular melanin in the mechanisms of active ion transport is discussed.

Animals↗
PubMed1985

[Passive immunotherapy in herpes zoster. A pilot study with intravenous hyperimmune globulin].

While passive immunisation with hyperimmune globulin proved beneficial for the prevention of varicella infections in immuno-compromised patients, there has been no reliable evidence for such preparations to be effective with the treatment of or prophylaxis against zoster complications. This may be partly due to the minor significance of the humoral immune response in herpes zoster as well as to insufficient bioavailability of the preparation when administered intramuscularly. In an openly and prospectively conducted study, a recently available varicella-zoster hyperimmune globulin was intravenously given to an unselected group of 20 in-patients suffering from herpes zoster. There have not been observed any side effects. If therapy was started within the first week of manifestation of the disease (15 test persons), the patients showed rapid and uncomplicated healing.

Adolescent↗
PubMed1984

[The value of diagnostic measures in erythema nodosum].

This article summarized the findings of check-ups of all our in-patients suffering from erythema nodosum during the past 15 years. A critical review showed that a third of the cases could not be etiologically defined. Based on our results and according to literature, we discuss the valence of diagnostic procedures in erythema nodosum patients without any history or physical evidence of underlying diseases.

Chronic Disease↗