Isoenzyme II of Escherichia coli L-asparaginase (L-asparagine amidohydrolase, EC 3.5.1.1) is among the few enzymes of major therapeutic importance, being used in the treatment of acute lymphoblastic leukemia. We have constructed several inducible expression systems that overproduce asparaginase II from recombinant plasmids. The most efficient of these systems consists of plasmid pTWE1, a derivative of pT7-7, and an ansB- strain of E. coli, CU1783. These cells produce and secrete amounts of asparaginase II that account for 10-15% of the total cellular protein. Most of the active recombinant enzyme can be released from the periplasmic space by a simple osmotic shock procedure. From the resulting material homogeneous asparaginase II was obtained by a two-step procedure. Overall yields of purified asparaginase were 10-15 mg asparaginase II per liter of E. coli culture. The recombinant enzyme appeared identical to conventionally purified preparations.

Amino Acids↗

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[Arthrogryposis, renal tubular dysfunction, cholestasis (ARC) syndrome: case report and review of the literature].

The ARC-syndrome is a rare disease with the obligatory symptoms arthrogryposis, renal tubular dysfunction and cholestasis. Optional further symptoms like ichthyosis, diarrhea, central nervous system defects and recurrent infections have been reported. The ARC-syndrome was first reported by Lutz-Richner and Landolt in 1973. The pathophysiology is still unknown, an autosomal recessive inheritance is postulated. Patients rarely exceed an age of six month. We report a boy of consanguineous Turkish parents who suffered from congenital deformities of the lower extremities, a metabolic acidosis and failure to thrive. In the sequel he developed a renal Fanconi syndrome and cholestasis. Histology of liver and muscle biopsy specimen showed the typical findings of the disease with giant cell hepatitis and neurogenous muscle atrophy. His condition could be stabilized and he increased in weight by substituting fluid, electrolytes, buffer and parenteral nutrition. Total enteral nutrition of the 280 ml/kg/d he required failed even by nasogastric tube and percutaneous endoscopic gastrostomy. Additional fluid substitution by central venous catheter remained necessary. At the age of 7 month he died.

Abnormalities, Multiple↗

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[Life-threatening theophylline intoxication: a variant of Munchhausen syndrome by proxy].

We report of a prematurely born infant, who was admitted to hospital at the age of 6 months due to seizures. The seizures continued despite of an improved electroencephalogram due to varying medications. The boy had episodes of hypokaliaemia, diarrhea, and tachycardia which were treated in critical care unit. Not before 3 months of continued treatment and diagnostic work up in three different hospitals had passed the underlying poisoning with theophylline by the mother was proved. The toxicity of theophylline is well known. Adverse reactions occur frequently during theophylline therapy. There are numerous reports of suicidal intoxications with theophylline. Non-accidental poisoning with theophylline has not yet been reported in the context of Munchausen syndrome by proxy.

Diagnosis, Differential↗

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[Necrotizing enterocolitis--analysis of a case cohort].

During a period of 25 months 11 newborns treated at the children's university hospital of Münster (0.8%) underwent laparotomy for necrotizing enterocolitis (NEC). The cases appeared sporadically without forming "clusters" typical of infectious etiology. 10 of 11 cases were premature infants (median birth weight 1280 g) stressing the predisposing role of immaturity. Ischemic events occurred in all patients prior to the onset of symptoms. The introduction of surfactant-therapy during the study period did not influence the incidence apparently. Ultrasonography was superior to radiology with regard to detection of extraluminal gas. We differentiated two types of clinical courses (3 acute and 8 protracted). In 5 cases we noticed a relapse of clinical symptoms after uptake of oral feeding. 3 newborns died with septic shock. The median hospitalisation time was 88.5 days in the 8 survivors. Enterostomy was replaced by definite anastomosis after 67.5 days (median). Median daily weight gain was 16.6 g. Late complications like permanent stenoses of short bowel syndrome were not observed. Early surgical intervention according to the general rules of abdominal surgery seems important even in very low birth weight infants.

Birth Weight↗

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Cystic fibrosis and metabolic alkalosis in children--revisited.

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Acute Disease↗

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[Unusual course of an advanced extra-uterine pregnancy].

We report about the course of an advanced ectopic pregnancy in case of uterine leiomyomata, which was diagnosed after surgery. A vaginal bleeding appeared in the first trimester. Vaginal ultrascan was missing in those early time of pregnancy. Finally prenatal diagnosis in a center of perinatal medicine led to a suspicious fetal morphology and gave the indication for medical abortion. The induction of abortion with Gemeprost remained without success. Meanwhile abdominal pain increased and a laparoscopic exploration was carried out and showed a big vital ectopic pregnancy. This indicated laparotomy. This case showed the difficulties in diagnosis of ectopic pregnancy in the second trimester and discusses possibilities of the therapeutic procedures.

Adult↗

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[The HELPP syndrome--a severe complication of pre-eclampsia. A presentation of 19 cases from 1983 to 1990].

Between 1983 and 1990, HELLP syndrome was diagnosed in 19 of 201 patients with preeclampsia. In addition to the characteristic changes in laboratory values, severe edema and proteinuria were found in almost all cases. Upper abdominal pain was the most common subjective symptom and, together with a reduced thrombocyte count, was often a first sign of HELLP syndrome. In some cases blood pressure was only slightly increased. Maternal and infant morbidity were significantly increased. Premature termination of labor was usually necessary, in most cases due to maternal pathology. The incidence of intrauterine dystrophy and premature delivery far exceeded the norm. Early detection of HELLP syndrome is crucial, and interdisciplinary cooperation is a key factor in achieving this goal.

Blood Coagulation Disorders↗

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[Pregnancy and labor in a duplex uterus].

It is reported about the clinical development of pregnancy and birth of a patient with uterus duplex separatus. One abortion was followed by two normal pregnancies. This case shows that in case of complete double-malformation of the uterus the pregnancy can develope without complication. Because of reported obstetrical complications a primary cesarean section should be preferred.

Adult↗