Postnatal age at time of death in infants born at 24-32 weeks' gestation

Medical records of all 483 infants live-born at 24-32 weeks' gestation in our hospital during the years 1982-86 were reviewed in order to determine postnatal age at time of death for those who died in the first year after birth. Twenty-seven died from immaturity without receiving intensive care and 11 died from lethal congenital malformations. Eighty (18%) of the remaining 445 who received intensive care died: 31% on day 1, 45% on days 2-7, 12% on days 8-28 and 11% on days 29-365. The neonatal mortality rate for those receiving intensive care was 160/1000, and the postneonatal mortality rate was 20/1000. Delayed mortality was most common in infants of 26-27 weeks' gestation, with 20% (five) of their deaths occurring after 28 days. In each gestational age range, the majority of hospital admission days were occupied by survivors (24-25 weeks = 62%; 26-27 weeks = 91%; 28-29 weeks = 91%; 30-32 weeks = 99%) rather than non-survivors. Whilst postneonatal mortality is a significant concern, these data suggest that if infants born at less than 33 weeks' gestation are offered intensive care and survive the early neonatal period, the long-term outcome is more likely to be survival rather than delayed death. Furthermore, the majority of hospital admission days invested in such infants involves those who will be discharged home rather than those who will not.     

Paroxysmal nocturnal hemoglobinuria presenting as recurrent hemolytic uremic syndrome

Paroxysmal nocturnal hemoglobinuria (PNH) may present with acute anemia, thrombocytopenia and, if hemoglobin nephropathy or dehydration is present, azotemia. Thus PNH may be confused with the hemolytic uremic syndrome (HUS). Recurrent episodes, though common in PNH, are unusual in HUS. A positive acid hemolysis test can be used to differentiate between the two diseases.     

Protein, lactose and fat concentration of breast milk of mothers of term and premature neonates

The protein, lactose and fat concentrations in the milk of seven mothers giving birth at term and six mothers giving birth prematurely were determined. Samples were obtained during the first 31 days of lactation. The protein, lactose and fat concentrations of preterm milk were found to be similar to those of fullterm milk over this period. For both term and preterm groups, protein concentration decreased with increasing age, while lactose concentration increased. Fat concentration did not show a consistent pattern of change in the preterm group, but tended to increase with advancing age in the term group.     

The lens in hereditary hyperferritinaemia cataract syndrome contains crystalline deposits of L-ferritin

BACKGROUND/AIM: Hereditary hyperferritinaemia cataract syndrome (HHCS) is an autosomal dominant disorder characterised by elevated serum L-ferritin and bilateral cataracts. The ocular manifestations of this disorder are poorly studied. This study therefore sought to determine the origin of cataracts in HHCS. METHODS: L-ferritin ELISA, immunohistochemical and ultrastructural analysis of a lens nucleus from an HHCS individual. RESULTS: The HHCS lens L-ferritin content was 147 microg/g dry weight of lens compared with <16 microg/g for a non-HHCS control cataract lens. The cataract comprised discrete crystalline inclusions with positive staining with anti-L-ferritin but not anti-H-ferritin. CONCLUSIONS: This unusual finding of crystalline opacities in the lens may be unique to HHCS and is likely to result from disturbed metabolism of L-ferritin within the lens or an abnormal interaction between L-ferritin and lens proteins.     

A regional poison control system. Effect on response to hypothetical poisonings

Poisoning of young children frequently results in parents seeking help. Parents either telephone for poison treatment information or go directly to a physician's office, hospital clinic, or emergency room. To determine if a regional poison control system reduces the inappropriate use of medical treatment services, parents attending suburban and inner-city clinics were presented hypothetical pediatric poisoning episodes and asked how they would respond. Parents living in a state serviced by a regional poison information center (Massachusetts) telephoned for information significantly more often and consequently used medical treatment services 19% less than did parents living in a state with only local poison information centers (New Jersey). In both states, inner-city populations went for care whereas suburban populations called. Both inner-city and suburban populations responded to a regional poison center but to a different extent. More severe ingestions tended to increase the use of the regional center by the Massachusetts suburban population, whereas other populations tended to use even more direct services.     

Autologous bone marrow transplantation in the treatment of children and adolescents with advanced malignant tumors

Nineteen patients with advanced malignant tumors, less than 20 years old were treated with intensive chemotherapy (vincristine 2 mg/m² i.v. and adriamycin 60 mg/m² i.v. on day ?7; cyclophosphamide 45 mg/kg i.v. on days ?6 to ?3), total body irradiation (TBI, 600 rads on day ?1) and autologous bone marrow transplantation (ABMT, day 0). Prior to this procedure induction of complete or partial remission by conventional therapy was attempted. Ten patients had intra-abdominal non-Hodgkin's lymphoma (NHL); three, yolk sac tumor; three, Ewing's sarcoma; and three, neuroblastoma. The supportive care included reverse isolation, immunoglobulin 400 mg/kg i.v. q 2 weeks, cotrimoxazole per os, and cell support as needed. No correlation between the bone marrow dose and the time of hematological reconstitution could be established. Five of seven patients with intraabdominal NHL stage III (transplanted in first remission) are surviving disease-free for 5 +, 5 +, 20 +, 23 +, and 35 + months after ABMT. None of three patients with intra-abdominal NHL stage IV is surviving (two of them were transplanted in second remission). One of three patients with yolk sac tumor is surviving disease-free for 27+ months. There are no survivors among the patients with Ewing's sarcoma and neuroblastoma. Only one of 19 patients was lost due to therapeutic complications, while 12 died due to tumor. Regarding treatment results for advanced intra-abdominal NHL, the procedure described here is comparable to the best conventional regimens. In vitro methods for tumor cell eradication in the collected bone marrow might further improve the results of ABMT.     

Torsade de pointes and asystole in a child with complete heart block and prolonged QT interval

Three-year-old girl with eight month history of convulsive syncope due to complete heart block, prolonged QT interval and paroxysms of ventricular tachycardia is described. An episode of torsade de pointes tachycardia followed by asystole was documented. The symptoms and electrocardiographic abnormalities were controlled by pacemaker therapy. The subset of "congenital heart block" patients with prolonged QT interval is at high risk of sudden death and should be regarded as a previously unrecognized form of Romano-Ward syndrome.     

Management of hepatic encephalopathy

Hepatic encephalopathy (HE) is seen as a clinical manifestation of low grade chronic cerebral edema, which is accompanied by alterations in glioneural communication. Different factors such as ammonia, inflammatory cytokines, benzodiazepines and electrolyte imbalances may precipitate or aggravate glia edema, thereby explaining precipitation of HE episodes by a variety of unrelated factors. Recognition and rigorous treatment of these precipitating factors is the most important measure in HE therapy which may be augmented by dietary and medical approaches. Among these approaches, evidence for proven therapeutical efficacy in HE on the basis of placebo-controlled trials is existing only for transplantation, protein restriction, administration of vegetable proteins, ornithine-aspartate, oral branched-chain amino acid treatment as well as lactulose enemas. The efficacy of oral lactulose has not been demonstrated on the basis of placebo-controlled trials.     

Transnasal surgery for prolactin-secreting pituitary adenomas in childhood and adolescence

BACKGROUND

Prolactin (PRL)-secreting pituitary adenomas (prolactinomas) are rare in childhood and adolescence; there are only a few published series of patients who were surgically treated. We discuss the gender-dependent differences, and the surgical indications and results for 14 patients with prolactinomas under 18 years of age at surgery.

METHODS

We reviewed the data for 14 patients (10 girls and 4 boys) treated surgically for prolactinomas between 1980 and 1996. Surgery was chosen because of intolerance and/or resistance to dopamine agonist (DA) in 9 patients, the patient’s decision in 3, rhinorrhea in 1, and pituitary apoplexy in 1 during DA therapy. All patients underwent direct transnasal explorations. Since 1988, intraoperative PRL measurements were performed. The follow-up period was at least 6 years.

RESULTS

Nine of the 10 girls had primary or secondary amenorrhea, and 3 of the 4 boys had visual field defects. The boys and girls were the same age at the onset of symptoms and had the same preoperative duration of symptoms. Boys had larger, more invasive tumors and higher preoperative and postoperative plasma PRL levels than girls. Two boys demonstrated significant extrasellar extension at the time of diagnosis. Two patients required drilling of the incompletely pneumatized sphenoid sinus to reach the sella. Radical tumor resection was achieved in seven girls and in none of the four boys. In 12 of the 14 patients, normal pituitary function was preserved by transnasal surgery. There was no severe surgical morbidity or mortality. Endocrinological remission by surgery alone was achieved for seven girls, and was achieved in 4 of the 5 patients (80%) with microadenomas. In patients with preoperative mean PRL levels of less than 200 μg/L, the surgical cure rate was 75%.

CONCLUSIONS

Prolactinomas in childhood and adolescence differ in their biology in boys and girls. Transnasal surgery is as safe in childhood patients as it is in adult patients, and it may be an effective alternative to long-term medical therapy for selected patients with prolactinomas. Successful surgical outcomes were achieved in patients with microadenomas and preoperative serum PRL levels of less than 200 μg/L.