[Severe, non-infectious mitral valve endocarditis after mitral valve reconstruction in a 32-year old female with primary antiphospholipid syndrome]. / Schwere, nicht-infektiöse Mitralklappenendokarditis nach Klappenrekonstruktion bei einer 32-jährigen Patientin mit primärem Antiphospholipid-Syndrom.

BACKGROUND: A 32-year old female with primary antiphospholipid syndrome presented 8 months after mitral valve reconstruction with progressive exertional dyspnea and echocardiographically demonstrable critical mitral stenosis and regurgitation. Tachycardia, weight loss, sleep disturbances and increasing nervosity led to the diagnosis of concomitant hyperthyroidism. After the patient stopped the oral anticoagulation by herself, a 'catastrophic antiphospholipid syndrome' with multiple microthromboembolic events in several organs developed rapidly within a few weeks. Severe respiratory failure was observed 14 days after admission at our hospital because of a pulmonary edema. TREATMENT: Removal of the annuloplasty ring and alloplastic mitral valve replacement with a 25 mm bilifleat valve. Postoperatively, the patient was placed on oral anticoagulation. Several pre- and postoperative plasmaphereses lowered the level of antiphospholipid antibodies. The patient additionally underwent radioiodtherapy 5 months postoperatively. RESULTS: While hemodynamics and diuresis remained sufficient, ventilatory support with tracheostomy was necessary for 16 postoperative days to achieve stable respiration. Thirty months later, the patient is well and without further cardiac and neurological dysfunction. CONCLUSION: Secondary cardiac valve operations on patients with primary antiphospholipid syndrome may be successfully performed within a multidisciplinary approach. Oral anticoagulation remains the treatment of choice to prevent further thromboembolic events.

Treatment of achalasia: botulinum toxin injection vs. pneumatic balloon dilation. A prospective study with long-term follow-Up.

BACKGROUND AND STUDY AIMS: In patients with achalasia, intrasphincteric injection of botulinum toxin (BTX) has been suggested as an alternative regimen to balloon dilation and has been shown to be superior to placebo injection. The aim of the present study was to test the effectiveness, the long-term outcome and the cumulative costs of BTX injection in consecutive patients with symptomatic achalasia in comparison with pneumatic balloon dilation. PATIENTS AND METHODS: 37 patients, who presented with symptomatic achalasia between January 1994 and December 1996 were treated with either BTX injection (n = 23) or pneumatic dilation (n = 14). Patients with short-term or long-term symptomatic failures of the initial procedure were treated again, either with the same or with the alternative method, depending on the initial response and on the patient's wish. Symptoms were assessed using a global symptom score (0 - 10) which was evaluated before treatment and 1 week, 1 month and then every 6 months after the treatment. In addition, body weight and recurrence of symptoms were noted and manometry was carried out before and after treatment. The patients were regularly contacted for the long-term follow-up. RESULTS: There were significant improvements in the global symptom scores of all patients treated, in both the BTX injection group (before 8.2 +/- 1.3, after 3.0 +/- 1.6) and the dilation group (before 8.3 +/- 1.1, after 2.3 +/- 1.9). There was also a significant decrease of lower esophageal sphincter pressure after treatment in the BTX group and the dilation group. There were no significant differences with regard to overall treatment failure and long-term outcome between patients who had or had not received previous treatment. No major complications were encountered in either group. An actuarial analysis over 48 months comparing patients receiving BTX injection or balloon dilation demonstrated that after 12 months neither therapy was significantly superior. After 24 months a single pneumatic dilation was superior to a single BTX injection, and after 48 months all patients treated by BTX injection had experienced a symptomatic relapse. In contrast, 35 % of all patients treated by dilation and 45 % of patients treated successfully by dilation were still symptom-free in an intention-to-treat analysis after 48 months. When the overall costs of treatment and further treatment after recurrence were compared, dilation and BTX injection showed a similar cost-effectiveness (costs per symptom-free day) after 48 months. CONCLUSIONS: BTX injection, which can be performed in an outpatient setting, is as safe and cost-effective as balloon dilation in symptomatic achalasia. Taking into account the lower long-term efficacy of BTX injection therapy, however, it is an alternative only in a minority of older or high-risk patients.

Autoantibody profile in progressive systemic sclerosis as markers for esophageal involvement.

We investigated the relationship between the severity and extent of esophageal involvement in patients with progressive systemic sclerosis (PSS) and the autoantibody profile. We studied 37 consecutive patients with PSS and compared their results to 25 healthy volunteers. Patients with PSS were separated into three subgroups: group 1 (antinuclear antibody [ANA] [+/-], anti-Sc170 antibody [Scl70] [-], and anticentromere antibody [ACA] [-]), group 2 (ANA [+], Scl70 [+], and ACA [-]), and group 3 (ANA [+], Scl70 [-], and ACA [+]). The lower esophageal sphincter pressure and the mean proximal esophageal amplitude were significantly lower in group 3 when compared with group 1, group 2, and the healthy controls. Distal esophageal aperistalsis was noted in 85% of group 3, 40% of group 2, and 30% of group 1. An involvement of esophageal motility was found in 100% of the patients with ACA. Our results suggest that esophageal involvement is more pronounced in patients with PSS with ACA as compared with patients with only Sc170 or ANA.

Albumin dialysis: effective removal of copper in a patient with fulminant Wilson disease and successful bridging to liver transplantation: a new possibility for the elimination of protein-bound toxins.

BACKGROUND: Acute liver failure may be the first manifestation of Wilson disease. If copper elimination fails, liver transplantation is the only remaining therapeutic option. Albumin dialysis, a new method for the removal of protein-bound toxins, was performed in a patient with fulminant Wilson disease. METHODS: An 18-year-old man with Wilson disease presented with hyperacute liver failure, hepatic encephalopathy III, oligo-anuric renal failure, haemolytic anaemia, rhabdomyolysis, pancreatitis and thrombocytopenia. He was treated with albumin dialysis using a 44 g/l albumin-containing dialysate and a slow dialysate flow rate (1-2 l/h). The other details of the technique used are similar to routine continuous veno-venous haemodiafiltration. RESULTS: One hundred and five milligrams of copper were removed by albumin dialysis within the first six treatments, resulting in normalisation of blood-copper levels. Successful treatment of the multiorgan failure was achieved. Hepatic encephalopathy improved within 2 days. The patient initially refused liver transplantation. Therefore 35 additional albumin dialysis treatments were performed. Forty-three grams of bilirubin (an indicator of detoxified substances in the liver) and 196 mg of copper were removed. Multiorgan failure, in particular hepatic encephalopathy, did not recur during 59 days of treatment. Eventually, the patient agreed to liver transplantation and that was successful. CONCLUSION: Albumin dialysis is a new method for the effective treatment of fulminant Wilson disease, resulting in the removal of protein-bound toxins copper and bilirubin. It may serve as a new treatment option in hyperacute liver failure of other origin, acting as an extracorporeal detoxifier.

Cholestasis induced hyperthyroidism after liver transplantation.

We report the case of a 68-year-old woman who underwent liver transplantation because of hepatitis-C-induced cirrhosis. She developed iodine-reduced hyperthyroidism postoperatively in temporal relation with cholestatic jaundice. Hepatic biopsies revealed moderate intrahepatic cholestasis and mild lobular inflammatory infiltration with some eosinophils. No histological evidence of acute graft rejection or reactivated hepatitis was found. Treatment with methimazole markedly reduced the serum parameters of cholestasis which, after subtotal thyroidectomy, returned to normal. Liver function recovered, as confirmed by repeated aminopyrine breath tests.

[Arterial vascular diagnosis by Doppler pulse curve analysis of the first dorsal metatarsal artery]. / Arterielle Gefässdiagnostik durch Doppler-Pulskurvenanalyse an der ersten dorsalen Metatarsalarterie.

In patients with peripheral arterial occlusive disease diagnostics by Doppler-sonography at the toe arteries and by Doppler-pressure measurement at the ankle arteries is often difficult and sometimes impossible. Using the first dorsal metatarsal artery for Doppler-waveform analysis yields exact information on haemodynamically relevant circulatory disturbances. Painful ulcers, gangrene, oedemata, mediasclerosis, burns, plaster casts, posttraumatic or postoperative conditions prevent the application of cuffs at the lower leg or foot. This Doppler-waveform analysis evidently represents the degree and the specialties of a disturbed blood circulation and allows an additional assessment of the vessels distal from the ankle arteries and enables a functional test on the anastomosis of the artery tibialis posterior and the artery dorsalis pedis. Functional and pharmacological influences on the tone of the vessels can be shown. The typical diagnostic features of Doppler-wave-form analysis used at the proximal measuring points are also valid at the dorsal metatarsal artery. The results of own comparative investigations in 69 patients and 44 healthy test persons are demonstrated.

[Metaphyseal chondrodysplasia of the McKusick type (cartilage-hair hypoplasia)]. / Metaphysäre Chondrodysplasie vom Typ McKusick (Knorpel-Haar-Hypoplasie).

Cartilage-Hair-Hypoplasia is a rare form of metaphyseal chondrodystrophia. Its clinical picture is characterized by dysproportionate deficient growth shift to length of upper part of the body. The hair diameter is reduced, and the eyebrows are defectly marked. After stimulation by insulin, the levels of somatotropic hormone are found in the acromegalic range. The bone structure is rarefied at the distal metaphyses of the metacarpals and the proximal metaphyses of the finger basal phalanges. The most important roentgenologic symptoms to be found are clowdy, cystic rarefactions at the distal femoral metaphyses. As to the pathophysiology, deficient proliferation of cartilaginous cells is mentioned in literature.

[Percentiles of the neck-girth in children and adolescents (author's transl)]. / Perzentilwerte des Halsumfanges im Kindes- und Jugendalter.

Correlative calculations as to the circumference of the neck in age-conformingly developed children have revealed that neck circumference exhibits the best correlations to body weight, followed by body surface, body height, age and cervical skin thickness. The graph of the 50. percentile demonstrates sex-different course with single discontinuities of level, which are characteristic in the beginnings of school-resp. of puberal ages. The positions of the 1-s-deviations beside the 50. percentiles are almost symmetrical. An essential result of this investigations is the correction of the wide spread opinion of an age dependency of neck circumference. Instead of this, nearly linear correlation exists between body weight and neck circumference.