Cryptorchidism (author's transl)]

The treatment of cryptorchidism should start at the age of two years because of pathological and anatomical studies. Ectopic testicles, inguinal hernias and postoperative situations associated with a wrong position of the testicle are primary indications for surgical corrections. Fixed inguinal, retractile or abdominal testicles may be primarily treated with human chorionic gonadotropin (HCG). The success rate after medical treatment averages 50%. The intranasal application of the releasing hormone of luteotropic hormone (LH-RH) may represent an alternative approach to the treatment of cryptorchidism. If treatment with LH-RH is to obtain the same success rate as HCG in larger series it may combine the advantage of painlessness with no androgenic side effects.     

Schimmelpenning-Feuerstein-Mims-syndrome (author's transl)]

The clinical picture of the Schimmelpenning-Feuerstein-Mims (or nevus sebaceus linearis) syndrome is described. The syndrome especially its excessive formes, is a relatively rare, but typical biotype of the neuroectodermal phakomatosis disorders. Symptomes are multiple widespread linear sebaceus nevi, seizures and mental retardation, ECG anomalies and ocular dysplasia and dystrophia, which can cause blindness.     

Immunosuppression (author's transl)]

Among different procedures of immunosuppression in man medical treatment with cytotoxic drugs has become the most important. Biological methods are still under development but promise to gain in importance since they are more specific and have fewer side effects. The clinical effect of immunosuppressive therapy in organ transplantation is undisputed. Its effects has been proven in some but not all immuno-inflammatory diseases. Before immunosuppressive therapy is started it must be known whether it is effective in the particular disease involved. The severity of the disease has to be critically balanced against the possible side effects of cytotoxic drugs.     

Incomplete membrane stenoses of the duodenum (author's transl)

A report in 2 children with incomplete membrane stenoses of the duodenum and further abnormalities in the gastrointestinal tract, the urogenital region and on the skeleton. In the first case ileus symptoms existed in early infancy following operated anal atresia. A duodenal diverticulum near the papilla was found with reflux of contrast medium in the biliary and pancreatic tracts. In the second case the clinical symptoms were limited; however, the x-ray findings of an intraduodenal pseudodiverticulum were impressive. Both patients were operated successfully by removal of the incomplete duodenal membrane. Both cases clearly showed the great variability in clinical symptoms with etiologically similar disease patterns and typical x-ray signs.     

Oculoauriculovertebral dysplasia (author's transl)]

A case of okulo-aurikulo-vertebral syndrome in a three year old girl is described. The similarities between the maxillary arch syndromes are emphasized on the one hand and on the other the relatively frequent occurrence of congenital unilateral facil palsy with congenital cardiac defects is outlined. Aetiological hypotheses are discussed.     

D-penicillamin-induced IgA-deficiency (author's transl)]

A 6 year old girl developed IgA-deficiency following treatment for chronic hepatitis with D-penicillamine. 4 months after discontinuation of the drug serum IgA and the number of circulating B-lymphocytes were still markedly reduced, in addition secretory IgA was diminished relative to secretory IgM. D-penicillamine should be used only if absolutely necessary and during treatment frequent monitoring of immunoglobulin levels becomes mandatory.     

Herpes simplex (author's transl)]

About 1-2% recurrent herpes simplex infections are seen in adults. In childhood, secondary herpetic infections are very rare. In contrast, primary infections by herpes simplex virus are of great importance: herpes disseminatus neonatorum (type 2), gingivostomatitis herpetica (type 1), eczema herpeticum (type 1; in some cases, also type 2) and keratoconjunctivitis herpetica (type 1). The clinical and therapeutic data are discussed.     

Overflow incontinence (author's transl)]

The encopresis of faecal impaction is a paradoxical incontinence found with constipation or faecal retention due to somatic or psychic causes. Normal continence comprises the possibility to delay passages of stools and the avoidance of leakage of stool in the meantime. Continued delay of defaecations causes faecal impaction and paradoxical leakage of stool. It is necessary to break the vicious circle by emptying the rectal ampulla and to avoid further constipation by means of laxatives and diet. Uderlying psychological disturbances must be recognized and treated. Two individual cases among our patients illustrate various aspects of the problem.     

Kryptophthalmus syndrome (author's transl)]

This peper describes the rare cryptophthalmus syndrome combined with several other malformations of the urogenital tract and of the nose, in addition to dyscephalia and cutaneous syndactilism of fingers and toes. Because of a high incidence of parental consanguinity autosomal recessive heredity is discussed.     

Reye syndrome (author's transl)]

Reye's syndrome is characterized by severe encephalopathy and fatty infiltration of the liver. Probably this is a polyetiological syndrome. In most cases the disease is preceded by influenza B or varicella infection. As known to date, damage to mitochondria is the essential feature. Therapy is symptomatic. A review is given of the clinical symptoms and of the many unsolved problems of pathogenesis.     

Secondary drowning (author's transl)]

Clinical features, pathophysiology, pathology and therapy of secondary drowning are described. This is a little-known complication of drowning accidents which needs intensive therapy, since it is often lethal.     

The monocyte-macrophage-system (author's transl)]

The importance of the monocyte-macrophage-system in specific and non-specific immune reactions is described. Macrophages are involved during both the afferent phase, e.g. antigen trapping, antigen processing, and the efferent phase, e.g. allograft rejection, tumour cell destruction, of the specific immune system. The monocyte-macrophage system can be activated by non-specific and - more important in vivo - by specific mechanisms via soluble factors produced by sensitized T-lymphocytes. Finally, some clinically important disorders of macrophage function are described.     

Asplenia and DIC (author's transl)]

3 cases of severe septic shock are described; a 5 month old girl with congenital hyposplenia, a 2 3/12 year old boy splenectomized because of microspherozytosis and a 11 6/12 year old boy splenectomized because of Hodgkin's disease. In 2 cases pneumococci were found in the blood cultures. In all 3 cases the coagulation analysis showed a consumption coagulopathy. Intravenous streptokinase treatment was applied in addition to general treatment for shock and antibiotic therapy. 2 patients survived and made a complete recovery, whereas the 2 year old boy died. The histological findings showed a severe DIC. In the Department of Surgery, Innsbruck, 44 children have been splenectomized during the last 6 years, 38 of whom we were able to follow up on for an average of 3.3 years. After an average of 1.2 years following splenectomy, 4 patients (including the 3 cases mentioned above) contracted acute septicaemia; a further patient also incurring a probable sepsis with DIC. 3 of these 5 children died, representing a morbidity rate of 13% and a mortality rate of 8%. The mortality rate is thus as high as that caused by the primary disease, indicating the urgency of prophylaxis for infections of this kind. 3 prophylactic forms of treatment are suggested: protection with penicillin, active immunization with polyvalent pneumococcal antigen and spleen preservation whenever possible.     

Dysplasia renofacialis (author's transl)]

Potter-syndrome with agenesia of the kidneys and typical face is only one variation of the big group of renofacial dysplasia. Among our patients which are under treatment because of malformations of the urinary tract very often we see children with dysplastic faces or malformed ears. Therefore vice versa patients with alterations of ears and face should be exactly investigated to exclude urinary tract malformations.     

Nursing and drugs (author's transl)]

Depending on their physiochemical properties, foreign substances appear to a more or less substantial degree in mother's milk. The decision as to whether a drug may be administered during nursing is particularly difficult in the case of antibiotics. Due to the development of resistance and sensitization only a few of these drugs are allowed. A nursing stop should only be ordered when a dangerous drug is used with genuine indication and without a possible alternative. As a rule, those drugs which are believed to involve a certain danger for newborn, can be replaced by other nonhazardous drugs.     

Cyclic agranulocytosis (author's transl)]

Report about cyclic agranulocytosis in a 10 1/2 years old girls, having stomatitis, sore throat and fever up to 39 degrees C in the interval of approximately 3 weeks since the age of 6 months. By the prophylactic treatment with pulverized tetracyclin, which is already given 2 days prior to the expected decrease of the neutrophils, the patient stays clinically asymptomatic since 7 months.     

Alport syndrom (author's transl)]

A deaf-and-dumb patient died at the age of ten of failure of the kidneys. An uncle of the boy died at the age of 42, an aunt at the age of 16 years, both of atrophic kidneys. The mother of the proband is suffering from hard hearing on one side and recurrent episodes of pyuria. Repeated examinations in two aunts revealed slight pyuria resp. erythruria. Contrary to most reports affected members of the family showed pyuria rather than haematuria. A special inheritance could not be proved.     

Burkitt's lymphoma (author's transl)]

Burkitt's lymphoma occurs endemically in Central Africa, but is also found sporadically in areas with temperate climate. A case of Burkitt's lymphoma found in our area is described. The literature in regard to the clinical symptoms, etiology and treatment of Burkitt's lymphoma is reviewed. At the present time cyclophosphamide is considered to be the drug of choice; it leads to a remission rate of about 90%.