[Kawasaki syndrome. Association with exposure to carpet shampoo and successful therapy with immunoglobulins in the second week of the illness]. / Kawasaki-Syndrom. Assoziation mit der Exposition von Teppichshampoo und erfolgreiche Therapie mit Immunglobulinen in der zweiten Krankheitswoche.

A male infant and a three year old girl, both with acute febrile illness, were admitted to our hospital for suspected meningitis/sepsis and gastroenteritis/severe viral infection, respectively. Both showed all six principal features of Kawasaki syndrome and revealed several other symptoms and laboratory findings commonly associated with the disease. The infant had multiple coronary aneurysms. The girl developed ascites, pancreatitis and iritis, all of which are seldomly recognized symptoms of the Kawasaki syndrome. The prompt and satisfactory therapeutic responses of both patients to the combined therapy consisting of oral acetylsalicylic acid (50-100 mg/kg b.w./d) and intravenous gamma-globuline (400 mg/kg b.w./d) at the eight and even eleventh day of illness support the use of gamma-globuline therapy beyond the first week of the disease. Prior to their illnesses both children had been exposed to carpet shampoo, an agent which has been repeatedly associated with an increased risk of Kawasaki syndrome.

Quantitative analysis for organic acids in biological samples: batch isolation followed by gas chromatographic-mass spectrometric analysis.

This new method for qualitative and quantitative determination of organic acids, aldehydes, and ketones in biological samples is effective for use with urine, plasma, and amniotic fluid, and it requires no deproteinization. Isolation by batch-wise liquid partition chromatography on silicic acid follows formation of the O-(2,3,4,5,6-pentafluorobenzyl)oximes of oxoacids, aldehydes, and ketones. The total organic acid content of the sample provides a rapid screening test for metabolic abnormality. A wide-bore, bonded-phase capillary column was used for quantitative gas chromatographic-mass spectrometric analysis, followed by automated identification and quantification. Analytical recoveries were quantitative for a wide variety of metabolites. Gas-chromatographic retention indices, discriminating ions, and control ranges in amniotic fluid, plasma, and urine of adult subjects were determined for 61 biologically important compounds.

High-frequency oscillatory ventilation combined with intermittent mandatory ventilation in critically ill neonates: 3 years of experience.

A heterogeneous group of 45 neonates with severe pulmonary disease and inadequate gas exchange on conventional intermittent mandatory ventilation (IMV) was treated with a high-frequency oscillator combined with an IMV (HFO-IMV) system (Emerson Airway Vibrator connected to a BABYBird 1 ventilator). The mean gestational age was 33 weeks (25.5-43) and mean birth weight 2.02 kg (0.66-4.24). Primary diagnoses included respiratory distress syndrome (RDS; 23), pneumonia (12), persistent fetal circulation (PFC; 6), diaphragmatic hernia/hypoplastic lungs (4). The IMV rate was reduced from 78 to 29 BPM (P less than or equal to 0.0005), while maintaining lower partial pressure of carbon dioxide (PaCO2) (P less than 0.005) and higher partial pressure of oxygen (PaO2) (P less than or equal to 0.0025). Active air leaks were present in 20 infants and these infants responded most favourably to HFO-IMV. HFO-IMV failed to improve ventilation in neonates with diaphragmatic hernia/hypoplastic lungs. Complications during HFO-IMV were increased pulmonary secretions (11), worsening or recurrence of pre-existing air leaks (11), or occurrence of new air leaks (10). In 4 patients death was related to major air leak complications. Twenty-four infants died, 18 of them of a respiratory cause. Twenty-one infants finally survived. We assembled a well-tolerated system to provide HFO-IMV and to successfully ventilate neonates with severe respiratory disease, who failed to respond to conventional IMV. Initiation of HFO-IMV earlier in the course of the disease in this type of infant may improve survival.

Skeletal anomalies in trisomy 21 as an example of amplified developmental instability in chromosome disorders: a histological study of the feet of 21 mid-trimester fetuses with trisomy 21.

In a previous radiographic study on the feet of 71 adults with trisomy 21 we found, in comparison to control individuals, an increased prevalence of biphalangeal toes and metatarsophalangeal sesamoid bones. The present histological study on the feet of 21 mid-trimester fetuses with prenatally diagnosed trisomy 21 confirms results of the earlier study. At both stages of development these minor bone anomalies have about the same frequency, thus suggesting 1) that they are selectively neutral, and 2) that they reflect a basic (innate) failure of ordered morphogenesis. Our observation that the normal spatial pattern of skeletal variants is reproduced in trisomy 21 simply on a quantitatively higher level lends sound support to the hypothesis of amplified developmental instability in chromosome trisomies.