Haemophilia A in two premature infants

The incidence of prematurity in Germany is about 10% and premature infants with haemophilia A (OMIM 306700) are in fact very rare. We report two new cases, one born in the 28th gestational week, weighing 1200 g with a factor VIII (FVIII) level of 0.03 IU/ml treated with bolus injections of plasma derived FVIII concentrate (pdFVIII), and one born at week 30, weighing only 710 g with a factor level of <0.01 IU/ml and treated with recombinant FVIII concentrate (rFVIII). Recovery of FVIII was 96% in case 1 and 120% in case 2, FVIII half-life was 6 h and 8 h, respectively. During FVIII substitution, neither bleeding, thrombosis nor inhibitor development were noted in both infants. Conclusion:Immediate and frequent factor VIII substitution appears to be safe and effective for prophylaxis and treatment in premature haemophilic neonates.     

Nutritional state and herniorrhaphy in premature infants

The incidence of inguinal hernia in premature infants is significantly high, and the optimal timing for its correction is controversial. Furthermore, whether the nutritional status of neonates will alter hernia diagnosis and postoperative results has not been demonstrated. The aim of this study was to analyze the results of premature neonates who underwent inguinal hernia repair before hospital discharge, comparing clinical features and perioperative data of two distinct nutritional groups. It was verified that early inguinal hernia repair is feasible without an escalation in the incidence of complications, thereby reducing the risk of incarceration and avoiding subsequent hospitalization. Moreover, premature infants had similar postoperative outcomes regardless of their nutritional status.     

Neonatal goiter with congenital thyroid dysfunction in two infants diagnosed by MRI

It is essential to diagnose thyroid dysfunction at an early stage because congenital hypothyroidism (CH) represents one of the most common preventable causes of delayed physical and psychological development, including mental retardation. US evaluates the shape of the thyroid gland. CT and scintigraphic examinations demonstrate iodine metabolism of the thyroid, but there is associated radiation exposure. We encountered two cases of congenital goiter, in which MRI was very helpful in confirming thyroid dysfunction and was very useful in making an accurate diagnosis.     

Functional intestinal obstruction in premature infants

Functional intestinal obstruction in two sets of twins is described. In each case the smaller twin was affected. The diagnosis was a clinical one which was supported by radiological findings. The stools from these two infants had a lower than normal water content.     

The use of a synthetic skin covering as a protective layer in the daily care of low birth weight infants

Skin damage is often seen as a result of the use of adhesive ECG electrodes. The protective quality of a new synthetic dressing, Omiderm, was tested on 34 infants weighing 800–3850 g. In a controlled trial the amount of skin damage caused by heart monitoring electrodes was evaluated after 48 h in which Omiderm protected the infants' chest and after an additional 48 h without protection. Omiderm did not interfere with the heart monitoring process. Results showed significantly less damage in the protected areas. No toxic or allergic reactions were observed.     

Permanent and transient congenital hypothyroidism in preterm infants

Aim: Transient fluctuations in thyroid function are well recognized in preterm infants. We wanted to assess TSH variation in babies with transient and permanent congenital hypothyroidism (CHT). Methods: Whole bloodspot TSH data in preterm infants (<35 weeks; 2005–2010) were assessed, and infants with bloodspot TSH values >6 mU/L identified. Permanent CHT was defined as a requirement for thyroxine beyond 3 years of age. Results: A first TSH sample was obtained from 5518 infants (median gestational age, 32 w; range, 22–35), with a second sample obtained from 5134 infants (median gestational age, 32 w; range, 22–35). Five infants had raised TSH concentrations on both occasions. Three of the five infants had a serum TSH >80 mU/L on second screen but two came off thyroxine beyond 3 years of age. All preterm babies with permanent or transient hypothyroidism were detected by the first TSH cut‐off of 6 mU/L. Only one infant with a birth weight <1500 g remains on thyroxine treatment beyond 2 years of age. Conclusions: The incidence of permanent CHT in preterm infants is similar to term infants. Profound abnormalities of thyroid function can occur in preterm babies with transient hypothyroidism but both categories of hypothyroidism can be detected by a ‘once‐only’ TSH screening strategy with a relatively low cut‐off.     

早产儿湿肺的临床特征及治疗对策

目的　对上海市早产儿中心早产儿湿肺发生情况、临床特征进行调查分析 ,旨在提高对该病的进一步认识。方法　调查 2 0 0 2年 1月 1日～ 2 0 0 3年 12月 31日出生的活产早产婴儿 75 2例 ,临床表现湿肺症者 4 8例。回顾性总结分析该病的临床特点、严重程度及影响因素、治疗手段和预后。结果　早产儿湿肺发生率为 6 38% ,可发生在各胎龄段和体重段之间。临床表现以症状出现早 ,多有呻吟、青紫、反应较差、明显吸凹征等为特点。病情严重程度与心脏功能密切相关。严重者多需用机械辅助通气。单纯湿肺症预后良好。结论　早产儿湿肺发生率相当高 ,剖宫产分娩仍然是重要影响因素     

Patent ductus arteriosus in premature infants: A review of current management

Summary Problems related to the ductus arteriosus confront the pediatrician more than any other isolated cardiac defect. Due to increased awareness of the problems of prematurity, the reported frequency of patent ductus arteriosus increased threefold between 1970 and 1975. Aggressive therapy directed toward closure of the ductus is indicated within the first week of life in the very immature infant. Pharmacologic closure of the PDA with indomethacin is effective about half the time.This paper was part of the Ray C. Anderson Symposium.     

Severe bacteria-associated hemophagocytic lymphohistiocytosis in an extremely premature infant

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition with high mortality. We report an extremely premature girl, born in the 24th gestational week (BW 732 g), that during her second month developed a severe HLH subsequent to a Serratia marcescens septicemia, with hepatosplenomegaly, cytopenias, hyperbilirubinemia (mostly conjugated, total bilirubin 916 mumol/L), hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia (21266 mug/L), and elevated sIL-2 receptor levels. Genetic analysis revealed no PRF1, STX11 or UNC13D gene mutations. Treatment was provided according to the HLH-2004 protocol with etoposide, dexamethasone, and immunoglobulin, but no cyclosporin because of immature kidneys. She recovered fully from the HLH but developed a severe retinopathy as well as green teeth secondary to the hyperbilirubinemia. We conclude that secondary, bacteria-associated HLH can develop in premature infants, and that HLH can be treated with cytotoxic therapy also in premature infants. It is important to be aware of HLH in premature infants, since it is treatable.     

Transplacental passage of non-ionic contrast agents resulting in fetal bowel opacification: a mimic of pneumoperitoneum in the newborn

Although animal studies suggest that non-ionic radiological contrast agents do not cross the placenta, case reports have documented transplacental passage of the non-ionic monomers iohexol and iopromide in humans. We report a case of presumed transplacental passage of the non-ionic monomer ioversol resulting in fetal bowel opacification and mimicking pneumoperitoneum.     

Comparison of the effects of theophylline and caffeine on serum erythropoietin concentration in premature infants

Theophylline administration has been shown to attenuate erythropoietin (EP) production in adults; the effect of caffeine is not known. Our aim was to determine whether caffeine and theophylline had similar effects on EP production in the premature newborn. If caffeine was found to have a greater effect, this would influence prescribing habits. Fifty preterm infants (mean gestational age 28 weeks) who had clinically significant apnoea were randomized to receive theophylline (4 mg/kg then 2 mg/kg twice daily) or caffeine (10 mg/kg then 2.5 mg/kg once daily). The methylxanthines were continued at least until discharge from the NICU and the dosage altered to keep the levels within the therapeutic range. As an assessment of EP production, serum EP concentrations were measured. Blood for EP, haemoglobin, reticulocyte count, theophylline and caffeine levels was obtained prior to treatment and at least during weeks 3 and 7. There was no significant difference in the mean EP level in the two groups taken prior to treatment at a median age of 2 days of life. There were similar falls in haematocrit and haemoglobin in the two groups during the study period compared to pre-treatment values. At that time, however, the median reticulocyte count was higher in the caffeine compared to the theophylline treated infants (P < 0.05). This was associated with a rise compared to baseline (median 10.0–0.2 mU/ml) in the mean EP levels in the caffeine group and a decrease from a median of 10.1 to 8.3 mU/ml in the theophylline group, but the EP levels in the two groups at week 7 did not differ significantly. Conclusion These results suggest that caffeine does not have a greater impact than theophylline on EP production. Received: 14 April 1997 / Accepted in revised form: 10 September 1997     

Retrolental fibroplasia in premature infants with patent ductus arteriosus treated with indomethacin

Sixteen premature infants were treated with indomethacin, either orally or intravenously, in order to close a symptomatic patent ductus arteriosus requiring mechanical ventilation. Positive effect was obtained in 4 of the 10 orally treated patients and in 1 of the 6 with intravenous administration. Immediate side effects were impairment of renal function, abdominal distension, and restlessness.Although the oxygen administration was guided using a transcutaneous oxymonitor, three severe cases of retrolental fibroplasia occurred. As indomethacin is a nonselective inhibitor of all prostaglandins, it might also cause a constriction of the retinal arteries, and as such be a factor in the development of retrolental fibroplasia in small premature infants.     

牛磺酸治疗早产儿全静脉营养胆汁淤积的疗效

目的　观察早产儿应用全静脉营养后是否出现胆汁淤积 ,并探讨牛磺酸治疗胆汁淤积的效果。方法　将小于 33周的早产儿分为甲乙两组 ,均行全静脉营养 ,甲组应用牛磺酸治疗 ,乙组未应用牛磺酸治疗 ,并与大于 33周早产儿及足月儿对照 ,入院后第 1、7、14、2 1天采血测定丙氨酸转氨酶、直接胆红素、γ谷氨酰转肽酶。结果　入院后第 14、2 1天甲乙两组直接胆红素、γ谷氨酰转肽酶显著高于大于 33周的早产儿及足月儿 ,各时段甲乙两组 3项指标无显著差异。结论　较长时间全静脉营养可致胆汁淤积 ,牛磺酸对胆汁淤积治疗无明显效果 ,表明全静脉营养导致胆汁淤积原因的复杂性     

Upper gastro-intestinal perforation in premature,mechanically ventilated infants with oesophageal atresia and tracheo-oesophageal fistula

Three cases of upper gastrointestinal perforation affecting the stomach, duodenum, and jejunum, respectively, are described. Each occurred in premature infant with oesophageal atresia and tracheo-oesophageal fistula on ventilatory support. In each case there was a delay in diagnosis. All infants survived. The management options and current opinion on the treatment of this difficult problem are discussed.     

Severe apnoeas following immunisation in premature infants.

Four premature infants developed apnoeas severe enough to warrant resuscitation after immunisation with diphtheria, pertussis, and tetanus (DPT), and Haemophilus influenzae B (Hib). One required re-intubation and ventilation. Although apnoeas after immunisation are recognised, they are not well documented. It is time for further research to elucidate the best time to immunise such infants.     

Pentoxifylline reduces plasma tumour necrosis factor-alpha concentration in premature infants with sepsis

Increased plasma tumour necrosis factor (TNF) concentration correlates with mortality in sepsis. We suggested that pentoxifylline (PTXF), which is known to inhibit TNF production, may improve survival and attenuate clinical symptoms of sepsis in neonates. Plasma TNF levels were evaluated in 29 newborn infants with sepsis. Patients were randomly assigned into two groups, receiving PTXF in a dose of 5 mg/kg per hour for 6 h or placebo (saline), on 3 successive days. Both groups were subjected to the same conventional therapy. TNF was evaluated before and after PTXF or placebo administration on the 1 st and 3rd days of therapy. There was a statistically significant decrease in plasma TNF level in the PTXF group when the values before the first and after the last PTXF infusion were compared [mean: 671.5 pg/ml; SD: 438; med: 729.6 vs mean: 41.0 pg/ml; SD: 64.1; med: 11.5;P<0.000004]. In the placebo group, decrease was not significant [mean: 633.0 pg/ml SD: 488.6; med: 618.9 vs 246.9 pg/ml; SD: 243.9; med: 191.0]. A significantly higher plasma TNF level, evaluated after the last PTXF infusion, was found in the placebo group [246,9 pg/ml vs 41.0 pg/ml;P<0.001]. Only one of four infants with signs of shock in the placebo group survived, whereas all of five newborns with symptoms of shock in the PTXF group survived [P<0.04]. An increased incidence of metabolic acidosis [P<0.05], necrotizing enterocolitis [P<0.04] and renal insufficiency [P<0.05] was observed in infants in the placebo group.Conclusion PTXF inhibits production of TNF and may have therapeutic value in the treatment of premature infants with sepsis complicatea by shock.     

Precordial contrast echocardiographic detection of patent ductus arteriosus in small preterm infants

Summary Clinical detection of patent ductus arterious (PDA) remains an important and challenging problem in the small preterm infant with respiratory distress. In this study, PDA was diagnosed in 28 small preterms using an improved contrast echocardiographic method. In these infants, the injection of saline into the aorta generated echoes which were imaged at the pulmonary valve. This was accomplished using a conventional M-mode ultrasound transducer applied at the usual precordial position. Contrast echo studies were compared with the degree of ductal patency shown by single film aortography. Ductal patency was detected by contrast echo in 29 of 31 instances of aortographically proven PDA. Indirect echo indices commonly used for detection of PDA (cardiac chamber enlargement) may be limited since factors other than left-to-right shunt can cause cardiac enlargement in distressed small preterms. This direct contrast echo technique is an easily performed, sensitive, qualitative method for confirmation of the diagnosis of PDA in small preterm infants. This work was supported in part by grant 5507 RR05551-17 from U.S. Public Health Service, Bethesda, Maryland Presented to members of the Cardiology Section at the 20th Annual Meeting of the Society for Pediatric Research, April 29–May 2, 1980, San Antonio, Texas