Delayed Presentation of Cardiac Perforation After Modified Ravitch Pectus Excavatum Repair.

Pectus excavatum repair using a Nuss bar is a widely performed procedure and generally regarded to be quite safe. Rare catastrophic cardiovascular complications have been previously reported, by even the most experienced surgeons. These cases typically present with fulminant cardiogenic shock and have an associated high mortality rate.

Continuous vs thrice-daily ceftazidime for elective intravenous antipseudomonal therapy in cystic fibrosis.

Background/aim: We hypothesized that a continuous 24-h infusion of 100 mg/kg per day ceftazidime (treatment C) would result in equivalent or even superior anti-infectious efficacy in chronic Pseudomonus aeruginosa (PA) infection in patients with cystic fibrosis (CF) in comparison to the usual application of 200 mg/kg per day ceftazidime in three doses (treatment T).

Methods: This was a randomized crossover study comparing outcome after 14 days and 35 days. Tobramycin administered once daily (10 mg/kg per day) was administered concomitantly in both groups.

Acetylcysteine In Diabetes (AID): a randomized study of acetylcysteine for the prevention of contrast nephropathy in diabetics.

Background: Patients with diabetes mellitus (DM) are at increased risk of contrast-associated nephropathy irrespective of their baseline creatinine (Cr). We tested the efficacy of N-acetylcysteine (NAC) relative to hydration in unselected patients (irrespective of baseline Cr) with DM.

Methods: We conducted a randomized open-label study comparing hydration alone (combined oral and rapid intravenous hydration, n = 69) to NAC plus hydration (similar hydration protocol plus NAC 600 mg BID x 4 doses, n = 68) in diabetic patients (mean age 65 +/- 10 years, 65% men) undergoing elective coronary angiography.

Influence of the cyclooctadepsipeptides PF1022A and PF1022E as natural products on the design of semi-synthetic anthelmintics such as emodepside.

The 24-membered cyclooctadepsipeptide (CODP) PF1022A, the active metabolite of the fungus imperfectus Mycelia sterilia (Rosellinia sp.) isolated from the plant Camellia japonica in Japan, is described as a powerful broad-spectrum anthelmintic natural product with low toxicity in animals. Further CODPs such as PF1022B, C, D and E have been isolated from the same culture and their structures have been established.

Monitoring of 4-hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency.

We report the successful treatment using low-dose vigabatrin (21.5-34 mg/kg/day) of a 10-year-old girl with succinic semialdehyde dehydrogenase (SSADH) deficiency We verified that 4-hydroxybutyric acid (GHB) concentrations in serum, cerebrospinal fluid, and urine continuously decreased in parallel with significant clinical improvement. Our results suggest that GHB quantification in physiological fluids may be a useful laboratory parameter for monitoring efficacy of vigabatrin treatment in SSADH deficiency.

Transmission of herbicide resistance from a monoecious to a dioecious weedy Amaranthus species.

The genus Amaranthus includes several important monoecious and dioecious weed species, and several populations of these species have developed resistance to herbicides. These species are closely related and two or more species often coexist in agricultural settings. Collectively, these attributes raise the concern that herbicide resistance might transfer from one weedy Amaranthus species to another.

Exercise inhibits epithelial sodium channels in patients with cystic fibrosis.

The aim of this study was to determine the effects of a single exercise bout on luminal Cl(-) and Na(+) conductance in the respiratory epithelium of patients with cystic fibrosis (CF). In nine patients with CF and nine healthy control subjects, the transepithelial electrical potential difference (PD) of the nasal respiratory epithelium was recorded, first at rest and then during moderate-intensity exercise. Under both conditions, PD was first measured while superfusing the epithelium with isotonic saline.

Bilateral deficit during short-term, high-intensity cycle ergometry in girls and boys.

Peak torque during an isokinetic bilateral knee extension or flexion is usually lower than the summed peak torque of two unilateral contractions, and this phenomenon is designated as bilateral deficit (BD). The objectives of this study were to determine whether a BD also exists for more complex, alternating movements (e.g.

Mutation reports: epidermolysis bullosa simplex associated with severe mucous membrane involvement and novel mutations in the plectin gene.

We report a novel case of epidermolysis bullosa simplex with severe mucous membrane involvement and mutations in the plectin gene (PLEC1). The patient suffered from extensive blistering of the skin and oral and laryngeal mucous membranes. Electron microscopy of a lesional skin biopsy showed cleft formation within the basal cell layer of the epidermis.

Granulomatous cheilitis and Crohn's disease in a 3-year-old boy.

Granulomatous cheilitis and Crohn's disease are disorders of unknown etiology. There are case reports describing their coincidence and pointing out the necessity of ruling out systemic disorders once the diagnosis of granulomatous cheilitis is made. However, such reports are few and the causal association of both diseases is controversial in the literature.

Albumin, IgG, retinol-binding protein, and alpha1-microglobulin excretion in childhood.

Urine concentrations of two high molecular weight proteins, albumin and IgG, and two low molecular weight proteins, alpha1-microglobulin (A1M) and retinol-binding protein (RBP), were measured in 657 healthy children from birth to 18 years of age. The urinary levels of RBP and A1M suggest an age-dependent decline, whereas the levels of albumin and IgG show an uneven distribution. Reference values for albumin, IgG, A1M, and RBP for each group are reported.

Peutz-Jeghers syndrome is caused by mutations in a novel serine threonine kinase.

Peutz-Jeghers (PJ) syndrome is an autosomal-dominant disorder characterized by melanocytic macules of the lips, multiple gastrointestinal hamartomatous polyps and an increased risk for various neoplasms, including gastrointestinal cancer. The PJ gene was recently mapped to chromosome 19p13.3 by linkage analysis, with the highest lod score at marker D19S886.

Autoimmune hepatitis following hepatitis A virus infection.

A 7-year-old patient is reported who suffered from fatigue and jaundice due to chronic hepatitis. He had acquired hepatitis A virus infection in his community and communicated the disease to his German family 4 weeks later. While the other family members recovered from acute viral hepatitis A, the patient presented 10 weeks after the onset of hyperbilirubinemia (12 mg/dl) with the histology of chronic hepatitis, absence of markers for viral persistence, presence of autoantibodies against smooth muscle (1:320) and the asialoglycoprotein receptor (1:600), and marked hypergammaglobulinemia (3700 mg/dl), leading to the diagnosis of autoimmune hepatitis.

[Specific gene deletion in patients with cystic fibrosis: pilot study of a small patient cohort].

Identification and molecular cloning of the cystic fibrosis (CF)-gene was a major progress in genetic counseling of families with one or more affected children. In caucasian families about 70% of the CF-patients show a homozygous or heterozygous deletion of one amino acid, that is phenylalanine at position 508 (delta F508). In a pilot study we examined the DNA of 14 CF-patients for F508 deletions.

[Severe liver involvement by Capillaria hepatica].

Human hepatic capillariasis is a rare disease, which predominantly affects children aged 1 to 4 years and has a poor prognosis in extensive infections. This is the first case observed in Germany. The main symptoms are those of severe parasitosis: persistent high fever, hepatomegaly, and excessive hypereosinophilia.

[Computerized perimetry in infants treated with ethambutol (author's transl)].

Computerized perimetry of the central visual field (Competer) was utilized in 12 eyes of children treated with Ethambutol because of tuberculosis to verify potential visual hazards of the drug. Visual acuity, visual field and the mean retinal threshold of the central field revealed no significant changes with increasing cumulative Ethambutol dose up to 166.5 g.

Continual observation of clinical and immunological parameters, in particular of salivary IgA, in tonsillectomised children.

In 106 tonsillectomised children the following parameters were measured before and after operation: clinical parameters (length, weight, postoperative complications), haematological parameters (BSR, Hg, PCV, RBC, and WBC), and immunological parameters (IgG, IgA, IgM, and salivary IgA); in a number of children measurements were taken over a period of more than 3 years. Standard values of salivary IgA in relation to total salivary protein were determined in noninfected children of various age groups. Compared to the standard and initial values, we found some statistically significant changes in the serum immunoglobulin levels and in the salivary IgA, but no irreversible pathological decrease in the above-mentioned parameters.

[Continual observation of clinical and immunological parameters, in particular of salivary IgA, in tonsillectomised children (author's transl)].

In 106 tonsillectomised children the following parameters were measured before and after operation: clinical parameters (length, weight, postoperative complications), haematological parameters (BSR, Hg, PVC, RBC and WBC), and immunological parameters (IgG, IgA, IgM and salivary IgA); in a number of children measurements were taken over a period of more than three years. Standard values of salivary IgA in relation to total salivary protein were determined in non-infected children of various age groups. Compared with the standard and initial values we found some statistically significant changes in the serum immunglobulin levels and in the salivary IgA, but no irreversible pathological decrease in the above-mentioned parameters.