[Congenital pulmonary fibrosarcoma. Differential diagnosis of infantile pulmonary spindle cell tumors]. / Kongenitales pulmonales Fibrosarkom. Differenzialdiagnose infantiler pulmonaler spindelzelliger Tumoren.

Primary pulmonary mesenchymal tumors are rare causes of intrathoracic lesions in newborns. We describe a case of pulmonary spindle-cell tumor with features of infantile fibrosarcoma and discuss the differential diagnosis of spindle-cell lesions in this location. In view of further case reports of the literature, this neoplasia can best be categorized in a spectrum of fibroblastic/myofibroblastic differentiated spindle-cell tumors, with excellent prognosis. Especially in congenital lesions a favorable clinical course is to be expected after complete surgical resection. Additional radio- and/or chemotherapy is not recommended.

Polyvinylchloride infusion lines expose infants to large amounts of toxic plasticizers.

PURPOSE: The purpose of this study was to evaluate whether infusion lines are able to leach plasticizers in substantial amounts and thus be a candidate substance for hepatotoxic effects during long-term total parenteral nutrition (TPN). METHODS: TPN solutions, blood products, and selected drugs typical for preterm infants concerning amount, content, and infusion time were perfused through common polyvinylchloride (PVC) infusion lines. Concentration of diethylhexyl-phthalate (DEHP) before and after perfusion was determined by gas chromatography/mass spectrometry. RESULTS: Daily quantities of DEHP by 24-hour infusions were Lipid emulsion 20%: 10185.6 microg; aminoacid/glucose-solution: 116.2 microg; midazolaminfusion for sedation: 26.4 microg; fentanyl for sedation: 132.5 microg; propofol for sedation: 6561.0 microg. The amount of DEHP by single doses of blood products (20 mL) were packed red blood cells: 144-608 microg; platelet rich plasma: 928 microg; and fresh frozen plasma: 552-8108 microg. The dose of DEHP for a typical preterm neonate requiring TPN and additional therapy like sedation or blood products is at minimum 10 mg and can easily reach 20 mg/d. CONCLUSION: This large amount of DEHP is especially disturbing, because it effects the most vulnerable patients (neonates). Whether there is a relation to TPN-induced hepatobiliary dysfunction remains to be elucidated and is under investigation. With respect to recent literature, a biological effect of these doses must be assumed.

Toxaphenes and chlorinated naphthalenes in adipose tissue of children.

BACKGROUND: Chlorinated hydrocarbons are ingested by humans in food and accumulate in adipose tissue. At the University Kinderklinik, Mannheim, previously unknown substances have been found in children (e.g., the pesticide toxaphene and chlorinated naphthalenes). These substances have been widely used for industrial purposes in the past. Samples from West and East Germany; Saratov, Russia; and Almaty, Kazakhstan were examined to determine whether these substances are ubiquitous. METHODS: After Soxhlet extraction, the extracts were cleaned up using a liquid chromatographic technique. Measurement was performed by gas chromatography-mass spectrometry using negative chemical ionization in the single-ion-monitoring mode. RESULT: In specimens from all cities, toxaphene congeners Parlar 26 and Parlar 50 and six chlorinated naphthalenes were traced. Highest median load of toxaphene was 1.97 microg/kg for Parlar 26 and 2.36 microg/kg for Parlar 50 in Stralsund, East Germany. For chlorinated naphthalenes, the median was highest in Mannheim, West Germany, with 12.0 microg/kg. CONCLUSION: These findings show that monitoring these toxic substances remains necessary. Even though the use and as a consequence the amount of chlorinated hydrocarbons were reduced, these substances have by no means disappeared from the environment.

Fibrinogen heterogeneity in homozygous plasminogen deficiency type I: further evidence that plasmin is not involved in formation of LMW- and LMW'-fibrinogen.

Human plasma fibrinogen is heterogeneous in SDS-polyacrylamide gel electrophoresis and other methods for separation of proteins by molecular size. A high molecular weight fraction (HMW-fibrinogen, 340 kD) contributes approximately 50% of total fibrinogen antigen. Low molecular weight fibrinogen (LMW-fibrinogen, 300 kD) adds another 40%. The residual amount is LMW'-fibrinogen with a molecular weight of 280 kD, and a small amount of very high molecular weight fibrinogen (Fib420), the product of alternative splicing of the A alpha-chain genetic information, resulting in an extended A alpha-chain C-terminus. Fibrinogen was detected by specific immunostaining of nonreduced SDS-PAGE gel immunoblots with antibodies against fibrinopeptide A. Using densitometric scans of the immunoblots we found a ratio of HMW-, LMW- and LMW'-fibrinogen in a patient with homozygous plasminogen deficiency that was similar to the ratio found in immunoblots of plasma from healthy blood donors. Treatment with plasminogen concentrate resulted in a slight decrease of the proportion of HMW-fibrinogen, followed by an increase to 78%. The LMW'-fibrinogen band gained intensity initially, increasing to 11.9% of fibrinogen antigen 6 h after starting plasminogen infusion, but then dropped to levels below detection limit of the immunoblotting assay. LMW-fibrinogen remained constant during the initial 72 h of plasminogen treatment, then dropping to values in the range of 22-25% afterwards. The proportion of HMW-, LMW-, and LMW'-fibrinogen again reached the initial levels two weeks after starting treatment with plasminogen concentrate. We conclude that plasminogen is not involved in the limited proteolysis leading to formation of LMW-fibrinogen and LMW'-fibrinogen in the absence of a generalized fibrinolytic condition. Fibrinolytic activation may lead to the formation of fibrinogen degradation product X, which appears in a similar position as LMW'-fibrinogen in SDS-PAGE.

[Chlorinated carbohydrate content of fetal and pediatric organs and tissues]. / Gehalt fetaler und kindlicher Organe und Gewebe an chlorierten Kohlenwasserstoffen.

Organic halogens are used as pesticides and in chemical industries. They are secreted with breast milk and accumulated in fat tissue of infants. Organic halogens can be found already in newborns. We analysed polychlorinated biphenyl (PCB), DDT, hexachlorocyclohexane (HCH), and heptachlor in subcutaneous fat tissue and other tissues (placenta, liver, kidney, lung, brain, thymus, muscle, heart) of 34 fetuses and dead children. These substances were found regularly in placenta, in fetal subcutaneous fat tissue and in fetal organs. They therefore can influence possibly early and sensitive stages of intrauterine development. The average concentrations found in fetal fat tissue were: PCB 0.7 mg/kg fat tissue, DDT 0.7 mg/kg, HCH 0.14 mg/kg, and heptachlor 0.03 mg/kg.

Chlorinated hydrocarbons in human bone marrow of healthy individuals and leukemia patients.

In this pilot study, concentrations of chlorinated hydrocarbons in bone marrow were determined by capillary-column gas chromatography. Bone marrow was obtained from a total of 29 healthy adults and from patients with leukemia or lymphoma. The chlorinated hydrocarbons in adults that occurred in the highest concentrations were dichlorodiphenyltrichloroethane and its derivatives (mean = 129 mg/g DNA). Hexachlorobenzene, the hexachlorohexane isomers, and dieldrin were also found, but in reduced concentrations (i.e., mean concentrations: 49.5 mg/g DNA, 14.9 mg/g DNA, and 5.9 mg/g DNA, respectively). Patients who had leukemia and lymphoma did not have significantly increased concentrations of chlorinated hydrocarbons.

A comparison of the concentrations of certain chlorinated hydrocarbons and polychlorinated biphenyls in bone marrow and fat tissue of children and their concentrations in breast milk.

Chlorinated hydrocarbon (CHC) and polychlorinated biphenyl (PCB) concentrations in the bone marrow of 57 children were compared with the concentrations in adipose tissue of 50 children and the concentrations in breast milk in the Federal Republic of Germany from 1984 to 1991. The concentrations of hexachlorobenzene (HCB), the dichlorodiphenyltrichloroethane (DDT)-metabolites, and polychlorinated biphenyl (PCB) congeners no. 138 and no. 153 were increased threefold, while the concentrations of several hexachloro-cyclohexane (HCH)-isomers and PCB congener no. 180 were only increased twofold. Because breast feeding is the primary source of CHC and PCB in toddlers and infants we also compared the concentrations in bone marrow of children with the concentrations in breast milk and found approximately fourfold higher concentrations for the most highly chlorinated PCB congener no. 180, but only threefold higher concentration for PCB 138 and 153 and the DDT-metabolites. The concentrations of beta-HCH and HCB were only slightly higher in bone marrow.

[Simultaneous determination of inorganic anions in body fluids. A new method in pediatric laboratory diagnosis--results of a pilot study]. / Simultane Bestimmung anorganischer Anionen in Körperflüssigkeiten. Eine neue Methode in der pädiatrischen Labordiagnostik--Ergebnisse einer Pilotstudie.

The anions analysis was a methodical problem up to now. This was the reason for low interest. Biological fluids like saliva and urine which could easily receive without any stress for the children, are little investigated for its capacity on nitrite, nitrate, bromide and sulfate. In this performance there will presented an ion-chromatographic method to determine inorganic anions in the following body-fluids: serum saliva, liquor and urine. The anions chloride, nitrite, bromide, nitrate, phosphate and sulfate was determined quantitatively. The method was proved in a pilot-study on children's body-fluids serum, liquor and saliva. The objects was to get a landmark in expectation from anion concentrations. Bromide was detected as a constant part in all body fluids. The origin and importance is not clear till now. Also was found nitrate in all investigated body fluids. There seems to be a connection between diarrhea and an increase in serum levels from nitrate. We found considerable amounts of nitrate in saliva by babies and infants. The method is distinguished by little fluctuation in measurement and high specificity. Short time in analysis and simple handling will do the method for a qualified one in pediatrics.

A pilot study on polychlorinated biphenyl levels in the bone marrow of healthy individuals and leukemia patients.

In this pilot study the concentrations of polychlorinated biphenyls (PCBs) was determined by capillary column gas chromatography in bone marrow from 29 adults. The highest concentration in all adult individuals was detected for PCB no. 180 (mean = 0.991) followed by two other highly chlorinated PCBs, no. 153 (mean = 0.918) and no. 138 (mean = 0.927). The less chlorinated PCBs, no. 101 (mean = 0.255), no. 52 (mean = 0.161), and no. 28 (mean = 0.324) contributed to a lesser extent. Additional samples from children (N = 19) were used to assess the dependence of PCB concentrations on patient age (Scheele et al. Eur J Pediatr 1992; 151:802-805). When comparing the data of adult leukemia and lymphoma patients with a reference group of healthy adult individuals, no significant increase in the leukemia patients was found.

Chlorinated hydrocarbons in the bone marrow of children: studies on their association with leukaemia.

Concentrations of chlorinated hydrocarbons and polychlorinated biphenyls (PCB) were determined by capillary column gas chromatography in samples of bone marrow from 38 children with leukaemia (16 samples/pools) and 15 control (5 pools). The highest mean and median concentrations were detected for total PCB (mean = 3.568 mg/kg fat basis/median 2.904 mg/kg) followed by the sum of the dichlorodiphenyltrichloroethane metabolites (1.775/1.059 mg/kg), hexachlorobenzene 0.354/0.260 mg/kg), the sum of the hexachlorocyclohexane isomers (0.133/0.093 mg/kg) and dieldrin (0.109/0.063 mg/kg). The CHC and PCB concentrations in bone marrow were two- to threefold higher than in fat tissue. Comparing children with and without leukaemia similar concentrations of CHC and PCB were found.

[Exposure of our children to pesticides, PCB and potentially critical anions]. / Belastung unserer Kinder mit Pestiziden, PCB und potentiell kritischen Anionen.

Among environmental pollutants the organohalogens still play an important role since they accumulate in human fat tissue and are secreted with mother's milk during lactation. Fortunately DDT-, HCH- and HCB-levels decreased in breast milk during the last years. In contrast, the PCB concentrations were still three-to five-fold above the permitted limits for cow's milk. Fat tissue of 262 newborns, infants and children was already as severely loaded with organohalogens as human milk. However, children with malignant tumors or congenital malformations did not show elevated concentrations in fat tissue. The significance of the potentially critical anions such as nitrate, nitrite, bromide, sulfate for the health of our children needs further clarification.

Chlorinated hydrocarbons in fat tissue: analyses of residues in healthy children, tumor patients, and malformed children.

The content of chlorinated hydrocarbons (CHC) was determined by means of capillary column gas chromatography in samples of fat tissue from 183 healthy children, 46 children with malignant tumors and 33 children with benign tumors or congenital malformations. The highest concentrations were found for total polychlorobiphenyls (PCB) (mean = 1.614 ppm), followed by the DDT group (mean = 0.556 ppm, HCB (mean = 0.097 ppm), the HCH isomers (mean = 0.083 ppm), dieldrin (mean = 0.020 ppm) and total heptachlor (mean = 0.010 ppm). Neonates displayed high concentrations in the adipose tissue before the first uptake of food. In the first six months of life, the concentrations of total PCB, the individual PCB components as well as DDT and HCB decreased significantly. In the second year of life, they rose again to the initial values and then remained relatively constant during the rest of childhood. The regional differences with regard to total CHC residues were slight, so that the CHC exposure cannot be reduced by a change of domicile within West Germany (FRG). Children with congenital malformations or benign or malignant tumors do not display raised concentrations of CHC.

[Follow-up of 60 children with ulcerative colitis and Crohn disease]. / Nachuntersuchungen bei 60 Kindern mit Colitis ulcerosa und Morbus Crohn.

The clinical courses of 60 children suffering from ulcerative Colitis (UC, n = 21) and Crohn's disease (CD, n = 39) were investigated over a period of 8.7 and 5.1 years respectively. At the time of diagnosis all UC-patients showed mucohemorrhagic feces. Relapses often occurred after emotional stress and in 81% of all patients during the winter season. Typical late complications were arthritis (6/21), osteoporosis (6/21), allergic diseases (8/21) and diabetes mellitus (2/21). However, the psychosomatic development appeared normal in at least 17/21 of these patients. Generally the courses in children with CD were more serious. Despite intensive therapy 15/39 children developed an intestinal stenosis which was followed by bowel resection in 11 of them. Further complications were fistulas (6/39), abscess-formations (4/39) and osteoporosis (12/39) due to steroid therapy. Only 12/39 showed a significant catch-up growth. Interviews and psychological tests revealed that CD-patients were introverted with strong connections to their families. Equally they longed for approval and social contact with their contemporaries.

[Course of chronic life-threatening digitalis poisoning in infancy with immunopharmacologic treatment using antidigoxin Fab of sheep]. / Verlauf einer chronischen lebensbedrohlichen Digitalis-Intoxikation im Säuglingsalter unter immunpharmakologischer Behandlung mit Antidigoxin Fab vom Schaf.

A 2 months old girl was given a tenfold increased dosage of Beta-Methyldigoxin for 2 weeks and subsequently developed severe symptoms of glycoside intoxication. In hospital she was treated by digoxin-specific Fab antibody fragments. 18 hours later the symptoms had totally disappeared. However, 48 hours from the beginning of the treatment free digoxin levels rose again to toxic ranges. In chronic intoxications the rediffusion of glycosides from tissues and interstitial space seems to be much more pronounced than in acute intoxications, and there is a higher risk of reintoxication.

[Late results following partial and total colectomy in infancy]. / Spätergebnisse nach partieller und totaler Colektomie im Säuglingsalter.

26 children were investigated on an average 11.5 years after partial (n = 13) and total (n = 13) colonic resection. Total colectomy was followed by an increased frequency of gastrointestinal symptoms such as recurrent abdominal pain, flatulence, attacks of diarrhoea, frequent and pasty or liquid stools with strange smell. An increased salt or fluid intake was observed in one half of these patients. Their height and bone age was slightly but significantly reduced. Laboratory investigations revealed no significant deficiencies of electrolyts, vitamins or trace elements. However Renin (mean and 2s-range = 5.2; 2.7-6.8 ng/ml.h, normal values (NV) 1.3; 0.5-4.0 ng/ml.h, p less than 0.02), aldosterone (242.1; 168.4-357.8 pg/ml, NV 78.9; 39.4-168.4 pg/ml, *p less than 0.02), conjugated bile acids (11.3; 5.2-20.0 mumol/1, NV 4.2; 1.5-7.0 mumol/1, p less than 0.01) and serum urea concentration (32.5; 20.8-48.7 mg/dl, NV 14.6; 6.0-22.5 mg/dl, p less than 0.01) were significantly elevated. Three postprandial plasma levels of gastrin, VIP and neurotensin were within normal limits. In patients with partial large bowel resection all signs were less pronounced. According to our results a special diet in children years after colectomy seems not to be required.

[Oligosymptomatic celiac disease--axis correction of extreme genu valgum with a gliadin-free diet]. / Oligosymptomatische Zöliakie--Achsenkorrektur eines extremen X-Beines unter gliadinfreier Kost.

We report on a girl who suffered from a severe left-sided Genu valgum. As there was a remarkable deterioration despite a conservative orthopedic therapy on osteotomy was planned. However, preoperative investigations revealed a malabsorption syndrome with osteomalacia due to coeliac disease. This gliadin-sensitive enteropathy had already been diagnosed and treated in infancy but had been ignored, since no abdominal symptoms had occurred after re-introduction of normal food. Four years after start of gliadin-free diet the false position of the left leg had disappeared and an operative correction had not to be performed.

[Toxicologic status in infant and child nutrition]. / Die toxikologische Situation auf dem Gebiet der Säuglings- und Kinderernährung.

Chlorinated hydrocarbon concentrations in adipose tissue of infants fed on mother's milk for 4 months are as high as those of their mothers after many years of deposition and accumulation. Both clinical experience and results of extensive experimental investigations with animal models indicate that these concentrations, which have decreased markedly in the last 10 years, are within the nontoxic range. Since these concentrations ought not be exceeded, it is advisable that, in nursing periods over 4 months, further loading be adapted to the increasing pesticide dilution occurring naturally in the tissue as the acquired residues are distributed in the growing body mass. A practical reference guide listing the allowable pesticide-dependent amount of mothers' milk per day for nursing periods exceeding 4 months was released by a Commission of Experts appointed by the Deutsche Forschungsgemeinschaft. By contrast, the heavy metal burden in infants fed powdered formula is higher than that in infants fed on mothers' milk. Since at least 10% of our household plumbing systems are made up of lead pipes and 75%, of galvanized iron pipes that contain lead, the heavy metals are acquired from the water used to prepare the formula. Older children consume cadmium and lead vegetable foods and organic residues primarily in animal foods. A complicated network of food ordinances and laws ensures that our children are not exposed to a general hazard; occasional consumer-related risks, however, cannot be excluded.

[Gastrin, secretin, VIP and motilin in children with mucoviscidosis and Crohn disease]. / Gastrin, Sekretin, VIP und Motilin bei Kindern mit Mukoviszidose und M. Crohn.

The number of peptide hormones which have been localized in the gut and in neurons of the central and peripheral nervous system has increased considerably. As there is almost no information about their importance in children with gastrointestinal diseases, we developed highly sensitive radioimmunoassays and measured postprandial serum/plasma levels of gastrin, secretin, vasoactive intestinal polypeptide (VIP) and motilin in 112 healthy children (N), 28 patients with cystic fibrosis (CF) and 17 children with Crohn's disease (CD). Gastrin values were not pathologic in children with CF nor those with Crohn's disease (N = 56.2 +/- 29.6 pg/ml; CF = 57.0 +/- 34.2 pg/ml; CD = 43.6 +/- 26.6 pg/ml). A significant age dependency was established for secretin and VIP. These peptides were elevated in CF-patients. In children with Crohn's disease only Secretin was increased. Motilin was elevated in all patients: N = 78.0 (49.1-124.0) pg/ml; CF = 148.0 (70.8-309) pg/ml; CD = 153.0 (87.6-266).

[Immunoreactive neurotensin in children with gastrointestinal diseases]. / Immunreaktives Neurotensin bei Kindern mit gastrointestinalen Erkrankungen.

Neurotensin is a 13-amino acid hormonal peptide, localized to specific areas in both the brain and gastrointestinal tract of many mammalian species. It has been described as causing a wide variety of central and peripheral actions. At present there is almost no information concerning the possible physiological and pathophysiological significance of this peptide especially in childhood. Therefore we investigated plasma concentrations of neurotensin-like immunoreactivity in 113 healthy and 103 children suffering from various gastrointestinal diseases. The fasting levels (48.7 +/- 17.4 pg/ml) were slightly lower (p less than 0.05) than our postprandial concentrations (72.9 +/- 28.1 pg/ml) 2-3 hours after breakfast. There was no age distribution. Significantly elevated levels were found in CF-patients (185.4 +/- 96.1 pg/ml; p less than 0.0001) and three children with active coeliac disease (252.3 pg/ml), whereas patients with Crohn's disease, ulcerative colitis and abdominal pain had normal values. Children after large bowel resection showed elevated plasma levels of neurotensin-like immunoreactivity (87.3 +/- 36.4 pg/ml), however, the differences were not significant.

[Anemic infarct of the liver following blunt abdominal trauma]. / Anämischer Leberinfarkt nach stumpfem Bauchtrauma.

Particularly in childhood, ischaemias of the liver are very rare. This is due to numerous collateral vessels and a dual blood supply of the liver. We, therefore, report on a 2-year old boy who suffered from an anaemic infarction of the whole left lobe of the liver after a fall. The clinical course and findings, including histology, are described. After resection of the left lobe the boy was free of symptoms.