A nationwide survey on transient hyperammonemia in newborn infants in Japan: prognosis of life and neurological outcome.

A nationwide survey of transient hyperammonemia in newborns was carried out in Japan. A total of 18 patients, consisting of 12 male and 6 female infants, were reported from 11 facilities. These neonates exhibited hyperammonemia with plasma ammonia levels in the range from 124 to 6256 micrograms/dl. Four newborn infants of the 18 died in the neonatal period, and an additional one died in the early infancy. Among the 13 infants who were alive at the time of this survey, 6 had neurological sequelae, including mental retardation, spastic quadriplegia and epilepsy. The multivariate analysis revealed that the Apgar score at 1 minute, peak plasma ammonia concentration, birth weight and sex were significant factors affecting the prognosis of life.     

Thyroid function in severely disabled children]

Thyroid function tests were studied in 105 severely disabled children. Fifty-four cases (53%) showed following abnormalities. Serum TSH concentration was increased in 2 cases. Both serum T4 and T3 levels were decreased in 18 cases (17%). Only the serum T4 level was decreased in 25 cases (24%). Only the serum T3 level was decreased in 10 cases (9.5%). Two patients showed primary hypothyroidism. Two patients (monozygotic twins) were suspected to have subclinical hypothalamic-pituitary hypothyroidism caused by septo-optic-dysplasia. Abnormal thyroid functions were caused by anticonvulsants in most patients. The serum T4 level was correlated with the number of anticonvulsants, but not correlated with motor performance (daily activity) or feeding function. Four patients who had low T4 level or low T4 and T3 levels were received L-thyroxine supplementation, but no clinical improvement was observed. The serum TSH concentration was decreased and the TRH test showed hyporeaction in all of these cases. The low T3 level and normal T4 level were not related to anticonvulsant administration. Two of these patients were in poor nutritional condition, resulting in so called low T3 syndrome.     

Use of small-bore silastic drains in general thoracic surgery.

STUDY OBJECTIVE: To evaluate the safety and efficacy of small-bore, silastic drains for patients undergoing general thoracic surgery. PATIENTS AND METHODS: Twenty-five patients who received soft, small-bore, silastic drains were compared with 17 patients who received semi-rigid double lumen (DL) tubes retrospectively. RESULTS: The pain score was significantly lower in patients who received the silastic tubes in video-assisted thoracoscopic surgery (VATS) cases on postoperative days 5 and 6 , after continuous epidural analgesia had finished (P=0.018). No specific morbidity was seen in the patients who received silastic tubes. CONCLUSION: We considered that soft, small-bore silastic drains were just as effective as traditional DL tubes, but caused less pain especially in VATS cases.     

Identification of secretin diastereoisomers produced during synthesis

The byproducts P-1 and P-2, which were produced during the synthesis of porcine secretin, were isolated in pure form from the crude secretin by HPLC. These were identified by a combination of amino acid analysis, enzymatic digestion, and isocratic or linear gradient reversed-phase (RP)-HPLC. The amino acid compositions of P1 and P2, determined by amino acid analysis after acid hydrolysis, were found to be the same as those of porcine secretin without distinction between L-and D-amino acids. But, HPLC of their digestive fragments with trypsin and alpha-chymotrypsin differed from that of secretin. The fragments, S7-12 of P-1 and S13-21 of P-2 were determined to be different from the corresponding fragments obtained from secretin by HPLC analysis of their digestive fragments. The amino acid composition of each acid hydrolysate, following digestion with D-amino acid oxidase, was found to have less leucine or alanine content than secretin. The HPLC analysis of the fragments from P-1 and P-2 by tryptic and alpha-chymotryptic digestion showed that they are the same as those from synthetic D-Leu10 secretin or D-Ala17 secretin, respectively. Consequently, P-1 and P-2 are concluded to be the secretin diastereoisomers, D-Leu10 and D-Ala17 secretin, respectively.     

ENDOSCOPIC MANAGEMENT OF A DIEULAFOY‐LIKE LESION IN A DUODENAL DIVERTICULUM

We present a 70‐year‐old man who had two episodes of melena during the preceding 8‐year period. He had a Dieulafoy‐like lesion in a diverticulum in the third portion of the duodenum. While emergency endoscopy revealed neither apparent blood nor clots around the diverticular orifice, there was a non‐bleeding vessel in the fundus of the diverticulum. The vessel ceased bleeding after argon plasma coagulation and, since then, the patient has not experienced bleeding. In cases of gastrointestinal bleeding of obscure origin, duodenal diverticulum should be considered as a possible source of bleeding, even when endoscopy discloses no apparent bleeding.     

An analysis of subretinal fluid in bullous retinal detachment

A patient with bilateral bullous retinal detachment underwent drainage of the subretinal fluid. The analysis of the subretinal fluid revealed a high protein concentration--17.4 g/dl OD, 27.5 g/dl OS--which was 3-5 times higher than that of the patient's serum. The protein fractionations of the subretinal fluid were almost the same as those of the serum. These findings may well explain the shifting fluid and support the theory that bullous retinal detachment is caused by the breakdown of retinal pigment epithelial function as a barrier between the choroid and retina.     

Three cases of spontaneous rupture of the oesophagus (Boerhaave's syndrome)

We reported three cases of spontaneous rupture which were surgically treated with direct suture method. One patient was operated on within eight hours after rupture with good results. In two patients diagnoses were made more than two days after rupture. One had esophageal fistula, but completely recovered after cervical oesophageal fistula, and another died of multiple organ failure. In this syndrome, early diagnosis and surgical treatment are essential for acceptable results. For early diagnosis, we emphasized on the presence of roentgenologic mediastinal and cervical emphysema. In patients for whom diagnosis was delayed and post-operative leakage at the oesophageal suture line occurred, temporarily fistulation of the cervical oesophagus proved to be effective in exclusion and diversion of the ruptured segment.