Plasmapheresis-induced remission in otherwise therapy-resistant FSGS.

We report an 8-year-old Caucasian boy who presented with steroid-resistant nephrotic syndrome. Renal biopsy showed the cellular variant of focal segmental glomerulosclerosis (FSGS). Within 1 year he received a series of therapies that have induced remission in other patients with this disease, all to no avail (conventional-dose cyclosporin A, methylprednisolone pulse therapy, high-dose cyclosporin A, and therapy with mycophenolate mofetil). He achieved remission after five sessions of plasma exchange. This case argues for aggressive therapy of resistant nephrotic syndrome in the native kidney. Plasma exchange should be considered as a possible rescue therapy arm in future study protocols.

Von Recklinghausen neurofibromatosis with urinary bladder involvement.

von Recklinghausen neurofibromatosis is an autosomal dominant transmitted disease with 100% penetrance but variable phenotypic expression. The incidence of this systemic disease is 1 in 3000 live births; however, genitourinary manifestations are rare. We report on our management of 1 case during the past 16 years.

Spiral CT of the lung in children with malignant extra-thoracic tumors: distribution of benign vs malignant pulmonary nodules.

The purpose of this paper is to clarify the distribution of benign vs malignant pulmonary nodules which are seen on spiral CT in children with malignant extra-thoracic solid tumors. Seventy-four children with known solid, extra-thoracic tumors underwent spiral CT of the chest. According to the initial and follow-up (interval 9.2+/-4.7 months) findings, the children were graded into four groups: I = normal; II = solitary nodule unchanged at follow-up; III = multiple nodules with one or more than one unchanged at follow-up; and IV = solitary or multiple nodules all changed at follow-up. Nodules without change at follow-up were regarded as benign. Forty-nine children did present with normal pulmonary CT exams. In 7 cases solitary pulmonary nodules were found unchanged (group II) at follow-up and in 2 cases (group III) some of the nodules were stationary. Thus, 12% (9 of 74) presented with at least one pulmonary nodule that did not change at follow-up. Solitary nodules (in groups II and IV) with a diameter <5 mm were in 70 % (7 of 10) unchanged at follow-up and regarded as benign. In children with known solid extra-thoracic tumors at initial presentation, 70% of solitary nodules ( <5 mm) may be benign. To avoid overstaging, smaller solitary nodules must not automatically be regarded as metastases.

[Unexpected regeneration of a congenital unilateral hypoplastic- dysplastic kidney after a contralateral nephrectomy for Wilms tumor]. / Unerwartete Regeneration einer einseitigen angeborenen hypoplastisch-dysplastischen Niere nach kontralateraler Tumornephrektomie.

The congenital hypoplastic-dysplastic kidney is characterized by a significant reduction of renal mass, an abnormal parenchymal differentiation and is associated with anomalies of the whole urinary tract. Not much is known about the ability for regeneration in such a kidney. We report on a 2 year 11 months old boy with an anaplastic Wilms tumor of the left kidney, in whom after tumor-nephrectomy an unexpected functional and sonographically documented morphological regeneration of the contralateral hypoplastic-dysplastic kidney occurred. The regeneration of this kidney is especially notable, because it occurred during nephrotoxic chemotherapy and radiotherapy.

Deacylation of influenza virus hemagglutinin does not affect the kinetics of low pH induced membrane fusion.

The relevance of palmitoylation of cysteine residues of influenza virus hemagglutinin (HA) for the HA-mediated membrane fusion triggered at low pH is investigated. Either wild-type HA (subtype H7) or mutant HA devoid of fatty acids were expressed in insect cells. The kinetics as well as the extent of fusion of HA-expressing cells with human erythrocyte ghosts were measured by a membrane mixing assay. Fusion was measured continuously at different pH by fluorescence dequenching of the lipid-like fluorophore R18 initially incorporated into the erythrocyte membrane. No significant difference between fusion of wild-type and mutant HA expressing cells with ghosts could be detected showing that deacylation does affect neither the extent nor the kinetics of fusion.

Assessment of fusogenic properties of influenza virus hemagglutinin deacylated by site-directed mutagenesis and hydroxylamine treatment.

Influenza virus hemagglutinin (HA) subtype H7 expressed from a baculovirus vector in insect cells requires cysteine residues for palmitoylation. Mutant HA devoid of fatty acids shows hemagglutinating and hemolytic activities almost identical to those of the acylated wild-type HA (wt). Using a membrane mixing assay (R18), neither the kinetics nor the pH dependence of fusion induced by wt or mutant HA was significantly different from virus-induced fusion. HA-induced fusion of insect cells with human erythrocyte ghosts could also be demonstrated by a cytoplasmic content mixing assay. Both species of recombinant HA induced the flow of lucifer yellow from preloaded ghosts into the cytoplasm of HA-bearing cells. This indicates that membrane fusion mediated by wild-type and fatty-acid-free HA includes both leaflets of the lipid bilayers. Hydroxylamine treatment of wt HA (H7) and fatty-acid-free mutant HA present in lysates of insect cells led to the complete inhibition of hemolytic activity. Deacylation of spike proteins by NH2OH treatment of virus particles resulted in a block of hemolytic activity in influenza virus subtypes H7 and H10 as well as of that in the togaviruses Semliki Forest and Sindbis virus. However, the same treatment did not affect subtypes H2 and H3 or two vesicular stomatitis virus serotypes. With such a differential effect whether or not fatty acids are present in the spike proteins of the different virus particles, hydroxylamine must have other effects than just deacylation, and therefore seems unsuitable for the study of the biological functions of acylproteins.

[Bone marrow transplantation for aplastic anemia--initial results in 8 patients]. / Knochenmarktransplantation bei aplastischer Anämie--Erste Erfahrungen bei 8 Patienten.

8 young patients (aged 11 to 23 years) with severe aplastic anaemia received bone marrow grafts from their HLA-identical, MLC-non reactive siblings. All patients had received repeated transfusions previously and had been unsuccessfully treated with corticosteroids (7 out of the 8 patients) and/or anabolic drugs (4 out of the 8 patients). In order to prevent graft rejection 5 patients received donor buffy coat cells after the marrow infusion and 3 patients underwent total body irradiation with 400 rad prior to the marrow transplantation. 5 patients are alive, 3 patients died. Death occurred from Candida septicaemia (day 4 after transplantation), left ventricular failure (day 14) and graft versus host reaction of the gut (day 85). The 5 living patients are in a very good state of health 30 to 166 days after transplantation. 4 patients already have normal blood cell counts. 2 of the surviving patients developed a transient GVH-reaction of the liver. One patient had a mild GVH-reaction of the skin on day 130.

[Results of antireflux-surgery in children according to Leadbetter-Politano]. / Operationsergebnisse der Antirefluxplastik nach Leadbetter-Politano bei Kindern.

The indications for operation and results of antireflux-operation according to Leadbetter-Politano method are discussed in a retrospective evaluation of 80 children. Age of child, grade of reflux, cystoscopic findings of ureteral orifices and therapeutic success in treatment of urinary tract infection are the main criteria for the indication to surgical intervention. The operation was successful in 79 out of 80 children. Three of them needed a corrective operation. The number of postoperative complications was in accordance with results in the literature. The incidence of postoperative recurrence of urinary tract infection in 7.5% was relatively low.

Is the first urinary infection in childhood a definite indication for radiological examination?

The need for X-ray investigations of the urinary tract at the time of the first proven urinary infection, even in older girls, is stressed. This policy is the best way to detect chronic pyelonephritis and congenital anomalies of the urinary tract and provide timely and appropriate treatment. These conclusions are supported by personal experience (see case description), and the incidence of congenital anomalies encountered during an 11 year period in our children's hospital. There were 957 concecutive cases with X-ray studies of the urinary tract (IVP and MCU). A considerable proportion (30%) were older children (4-12 years) having supravesical obstruction defined when the X-ray studies were performed at the first urinary infection. The proportion of abnormalities rose to 66% when the investigations were done after recurrent infections, which may contribute to the development and progression of disease.