The impact of peripartum factors on the onset and duration of lactation.

Knowledge of peripartum indicators of those mother-infant pairs that are at increased risk of early failure of lactation may improve specific support of breastfeeding. Mode of delivery, labor complications, hyperbilirubinemia, milk intake and weight development were evaluated in healthy term infants in a hospital (n = 338). Delayed onset of lactation was observed in primiparae and in study participants with peripartum complications. The quantitative intake of human milk, assessed by test weighing 0-24 h and 24-48 h after the onset of lactation, was not significantly different between these groups. In addition, volume intake, weight gain and lactation success were tracked in 77 infants. Partial feeding of infant formula or an intake of <150 g of human milk per day 24-48 h after the onset of lactation was linked to weaning within 4 weeks. Ninety-one percent of the infants were exclusively breastfed at discharge; this value had declined to 49, 35 and 20% at 4, 12 and 20 weeks, respectively. Peripartum factors may contribute to early lactation failure; the long-term success of breastfeeding was predominantly determined outside the hospital.

Molybdenum in infancy: balance studies in patients with intestinal stomata.

UNLABELLED: This study investigated the supply of the essential trace element molybdenum (Mo) in infants with preternatural anus. It was divided into two separate investigations: (A) the complete fecal excretion of nine patients was collected in fractions over 24 h; and (B) 72 h balance studies and parallel plasma analysis were conducted in five infants and complemented by the longitudinal comparison of one of these patients with his (otherwise healthy) premature sister. Atomic absorption spectroscopy (balance studies) and high-resolution inductively coupled plasma mass spectrometry (HR-ICP-MS, plasma) were used for analysis. The following results were obtained. (A) The fecal Mo concentration ranged from 1.98 to 42.02 nmol Mo g(-1) dry fecal weight. (B) The daily intake in the balance studies was 43.2 (11.33-100.5) nmol Mo kg(-1) and the median retention was -2.91 (-32.45 to 48.6) nmol Mo kg(-1). In the premature twins the boy with an intestinal stoma had a negative balance and lost -3.32 micromol within 32 d, while his sister retained +0.45 micromol Mo in the same period. Plasma Mo ranged between 9.4 and 46.7 nmol l(-1). CONCLUSION: The negative Mo balance results may indicate an increased risk of Mo deficiency in infants requiring a long-term preternatural anus.

Molybdenum balance studies in premature male infants.

UNLABELLED: Despite the fact that the trace element molybdenum (Mo) is essential, there is insufficient knowledge about the demands in infancy. Mo balances were therefore assessed under consideration of formula Mo concentrations ranging from 0.125 to 2.704 micromol/l. Sixteen premature male infants participated in the investigation. Their birth weights were between 1,500 and 1,990 g, the median (range) gestational age was 34 (32-36) weeks and the post-conceptual age at the time of study 37.4 (34.1-40.6) weeks. Twenty-four balance studies were performed and the materials analysed by atomic absorption spectroscopy. Infants with a "low" Mo intake received 0.024 (0.020-0.035) micromol/ kg per day, had a urinary excretion of 0.02 (0.008-0.045) and a retention of 0.0006 (-0.03 to 0.008) micromol/kg per day. Infants with a "high" intake received 0.284 (0.227-0.487) micromol/kg per day, had a urinary excretion of 0.243 (0.118-0.378) and a retention of 0.022 (-71.1 to 141.44) micromol/kg per day. Since the median urinary excretion exceeded 60% of the Mo intake at low and high intakes, sufficient resorption but minimal retention was assessed at low intakes of Mo. CONCLUSION: In view of the limited knowledge of long-term exposure to an elevated molybdenum intake and the substantial retention observed at higher intakes, upper limits should be set for molybdenum concentrations in preterm infant formulas.

Hereditary fructose intolerance and alpha(1) antitrypsin deficiency.

A patient with coexisting hereditary fructose intolerance (HFI) and alpha(1) antitrypsin deficiency (alpha(1)ATD) is described. Protease inhibitor typing was not conclusive, presumably because of impaired N-glycosylation secondary to HFI. The case underlines the diagnostic role of molecular genetic techniques in inborn errors of metabolism.

[Atrial ectopic tachycardia in infancy caused by tumor of the interatrial septum]. / Atriale ektope Tachykardie im Säuglingsalter durch Tumor des interatrialen Septums.

A case of atrial ectopic tachycardia in an infant probably caused by a small tumor in the interatrial septum is presented. The arrhythmia was successfully treated with amiodarone. Several histologic abnormalities are known to cause AET, but an intracardiac tumor detectable by two-dimensional echocardiography is extremely rare. AET -especially in childhood-as well as cardiac tumors show a tendency to regression, so that even in this case medical treatment should be the first therapeutic choice. Interventional or surgical treatment is required just for those patients with AET this is resistant to medical treatment or reoccurs after finishing the medical treatment.

The influence of mode of delivery on hematologic values in the umbilical vein.

A variable white blood cell count without clinical signs of sepsis or hematological disorders is often observed in newborns. The intention of this study was to investigate the effect of mode of delivery on hematologic measurements, especially white blood cell count in the umbilical vein. 121 term newborns were investigated prospectively. They were subdivided into three groups: spontaneous births (n = 83), vacuum extraction (n = 19) and cesarean sections (n = 19). The mode of delivery influenced the leukocyte count and the precursor cells. The mean leukocyte counts after spontaneous birth (14.6 +/- 4.0 leukocytes/nl) and after vacuum extraction (16.6 +/- 7.4 leukocytes/nl) were significantly higher than after cesarean section (12.1 +/- 4.4 leukocytes/nl; p < 0.05). The precursors of leukocytes were also significantly higher in infants with vacuum extraction (1.06 +/- 0.83 cells/nl) than in spontaneously delivered infants (0.54 +/- 0.47 cells/nl). An inverse relationship between the number of leukocytes and the arterial cord blood pH was observed (p < 0.05). No significant differences were observed in the effect of mode of delivery on erythrocyte and platelet counts.

[Tuberculous spondylitis--a forgotten differential diagnosis in backache]. / Spondylitis tuberculosa--eine vergessene Differentialdiagnose bei Rückenschmerzen.

A four-year-old boy had been complaining for one year of backache when sitting or bending. Radiological changes in the lumbar spine were at first interpreted as due to an old vertebral fracture. Half a year after the onset of the symptoms his sister was found to have open pulmonary tuberculosis, but a positive Tine test (he had not received BCG vaccination) was without any consequences. He was finally hospitalized five months later because the backache persisted and he now also had impaired movement of the left hip-joint. Physical examination revealed a 10 x 15 cm firm mass in the abdomen. Imaging demonstrated destruction of the 4th and 5th lumbar vertebrae and a large abscess. Acid-fast bacteria were found in gastric juice. Tuberculostatic treatment was started with isoniazid (10 mg/kg), rifampicin (10 mg/kg) and pyrazinamide (30 mg/kg). Two weeks later spondylectomy was performed, the defect being bridged with bone chips from the iliac crest. Four months after the operation, tuberculostatic treatment now being only isoniazid and rifampicin, he was able to partake fully in all activities of his age group.

Comparison of umbilical arterial versus umbilical venous blood pH correlated with arterio-venous glucose difference and cardiotocographic score.

The value of clinical parameters and umbilical arterial blood pH as indicator of prenatal hypoxia is disputed. In a prospective study of 86 vaginally delivered full-term infants, cardiotocographic (CTG) findings obtained 0-30 min and 30-60 min before birth were compared to pH values, O2 and CO2 partial pressures and glucose difference in umbilical arterial and venous blood. CTG findings were expressed as a score, higher values indicating fetal hypoxia. The venous but not the arterial blood pH was significantly related to the later (0-30 min) CTG score. The arterio-venous glucose difference was significantly related to both CTG scores. There was a significant statistical relationship between glucose difference and venous but not arterial blood pH. The later CTG score (0-30 min) also correlated significantly with O2 and CO2 partial pressures and base excess in the umbilical vein of all vaginally born infants. If CTG is accepted as an objective indicator of fetal hypoxia before birth, the arterio-venous glucose difference, and in the investigated range of pH-values, umbilical venous blood pH are more suitable than the arterial blood pH to ascertain the peripartal situation of the newborn.

Hirsutism-skeletal dysplasia-mental retardation syndrome with abnormal face and a uric acid metabolism disorder.

We report on a German boy, born to nonconsanguineous parents, with an apparently new combination of multiple congenital anomalies including extraordinary hirsutism, marked brachycephaly, abnormal position of thumbs, pedes excavati with claw-toes, an abnormal face, and mental retardation. Additionally the boy manifests a disorder of uric acid metabolism. This sporadic case may represent a new syndrome due to a fresh autosomal or X-linked mutation, or chance-isolated case due to segregation of an autosomal recessive gene.

Faecal excretion in infants.

Data on normal defaecation patterns in relation to diet during the first months of infancy are very limited. We therefore investigated in a prospective study faecal weight and gastro-intestinal passage time of breast fed (n = 12) and formula fed (n = 14) male infants. These were studied in 72 h collecting periods at the age of 17, 35, 57, 87 and 113 (+/- 4) days. Breast fed infants had a significantly lower daily dry faecal weight than formula fed infants in all periods investigated (median at the age of 113 days: 0.28 (0.17-0.75) g/kg and 0.81 (0.22-1.2) g/kg, respectively). Breast fed infants showed a large variation of gastro-intestinal passage time (6.79 h [range: 1.79-13.38 h] at the age of 17 days, 21.84 h [range: 5.41-75 h] at the age of 113 days). Comparable values of formula red infants were 13.75 h (range: 7.13-35.25 h) and 17.42 h (range: 5.38-36.5 h). Despite the efforts of approximation of infant formula to breast milk, differences of defaecation patterns in relation to diet are still relevant at this age and have to be considered in clinical practice.

Isolated defect of peroxisomal beta-oxidation in a 16-year-old patient.

We describe a 16-year-old boy suffering from psychomotor retardation, sensorineuronal hearing impairment, peripheral neuropathy, hepatosplenomegaly, short stature and delayed puberty. Postnatally, muscular hypotonia, mild facial dysmorphism and delayed fontanelle closure had been noticed. At the time of our examination, adrenal cortical function was normal. Biochemical analysis revealed accumulation of very long (> C22) chain fatty acids in plasma and fibroblasts. Furthermore, elevated levels of intermediates of bile acid synthesis and phytanic acid were detectable. These findings are consistent with a defect in the peroxisomal beta-oxidation system. A generalised defect of peroxisomal function was excluded by normal plasmalogen levels in erythrocytes and normal plasmalogen de novo synthesis in fibroblasts. Immunoblotting of the peroxisomal beta-oxidation enzymes gave normal results suggesting retained immunoreactivity but catalytic inactivity of one of the enzymes involved, probably either the trifunctional protein or the peroxisomal ketothiolase. This case markedly differs clinically from the few published reports on isolated deficiencies of peroxisomal beta-oxidation. Among the patients with comparable biochemical findings, this is the first report of survival into adolescence.

[Craniometaphyseal dysplasia--characteristic roentgen findings]. / Craniometaphysäre Dysplasie--Charakteristische Röntgenbefunde.

Craniometaphyseal dysplasia is a rare disorder of bone remodeling, which is characterized by hyperostosis or sclerosis of the skull combined with metaphyseal flaring of the long tubular bones. Diagnosis is only possible on the basis of the characteristic radiographic findings, these are shown in a case report of a 2 1/2 year old boy. There is no therapy of craniometaphyseal dysplasia.

[Sodium and potassium metabolism in infancy]. / Natrium- und Kaliumbilanzen im Säuglingsalter.

BACKGROUND: Recommendations for the concentration of most nutrients in infant formulas are based on their concentration in human milk. Industry succeeded in adapting sodium and potassium content in infant formulas to concentration found in human milk. Whether this adaptation affects on infants' mineral balances was studied in breast-fed and artificially-fed infants. METHODS: Sodium and potassium balances were performed in 16 term male infants from their 3rd until their 17th week of life. The balances were performed at home and comprised up to five periods in intervals of three to four weeks. Each balance period consisted of subsequent three 24 h collections of milk, stool and urine samples. Ten infants were breast-fed, six received an adapted infant formula supplemented with copper, zinc and iron. RESULTS: The breast-fed infants got a mean intake of 1 mmol Na/kg b w x day and a mean intake of 1.8 mmol K/kg b w x day. Man retention was 0.4 mmol Na and 0.7 mmol K/kg b w x day. The formula-fed infants received 1.9 mmol Na/kg b w x day and 2.1 mmol K/kg b w x day. Na- and K-retention in this group was 0.5 and 0.6 mmol/kg b w x day respectively. Although sodium intake in the formula-fed infants was nearly twice as much as in the breast-fed infants the difference in sodium retention was only small (0.4 vs 0.5 mmol/kg b w x day). The formula-fed infants got more potassium than the breast-fed infants, but potassium retention was the same in both groups. CONCLUSIONS: With the adapted infant formula of this study the artificially fed infant was as well supplied with sodium and potassium as the breast-fed infant. A further reduction of the sodium concentration seems not to be useful.

Longitudinal zinc balances in breast-fed and formula-fed infants.

Longitudinal zinc (Zn) balance studies were performed under domestic conditions in term breast-fed (n = 10), term formula-fed (n = 5; Zn concentration in the formula: 4 mg/l) and preterm formula-fed (n = 3) infants during the first 17 weeks of life. Samples of milk, urine and feces were analyzed by atomic absorption spectrometry. The median daily Zn intake in breast-fed infants decreased from 0.592 (0.457-0.829) mg Zn/kg body weight to 0.151 (0.095-0.304) mg Zn/kg body weight in the first 17 weeks of life; comparable values for bottle-fed term infants were 0.58 (0.511-0.701) and 0.674 (0.529-0.731) mg Zn/kg body weight. The median percent retention of Zn intake was 27 (-60 to 81.4)% in breast-fed infants and 21.5 (-42 to 64)% in formula-fed infants. In view of the urinary and fecal Zn losses measured, a daily intake of 0.3-0.5 mg Zn/kg body weight is considered to be sufficient to ensure a Zn retention equivalent to breast-fed infants. This requires a Zn concentration of 2-3 mg/l of Zn depending on milk volume intake.

Iron availability from an infant formula supplemented with bovine lactoferrin.

Iron balance studies were performed in 16 term infants from their 3rd until their 17th week of life. The balance studies were performed at home and comprised five periods with an interval of 3 to 4 weeks, each consisting of three 24-hour collections of milk and stool samples. Seven infants were fed an adapted infant formula supplemented with bovine lactoferrin (100 mg/100 ml) and nine received the same formula without lactoferrin. The lactoferrin supplemented group received 169 micrograms iron/kg b.w. x day and retained 63 micrograms/kg b.w. x day. The mean iron intake of infants fed with the adapted formula without supplementation of lactoferrin was 118 micrograms/kg b.w. x day. The retention of iron was 43 micrograms/kg b.w. x day. Mean percentage retention of iron in the supplemented group was 36%, in the non-supplemented group 28%.