Narcotic withdrawal syndrome following gastric bypass--a difficult diagnosis.

BACKGROUND: It is common for bariatric patients to experience postoperative nausea, depression and remorse for several months following surgery. Difficulty exists for the surgeon in delineating the physical from the psychological in treating these patients. Preoperative education, evaluation and preparation, although essential, will not identify nor eliminate all potential problems. METHODS: We report the case history of a patient who ultimately underwent reversal of her gastric bypass. Her symptoms required multiple procedures and hospital admissions for what appeared to be anatomical problems. All procedures were done laparoscopically. RESULTS: The patient's main complaints of persistent nausea and abdominal pain combined with radiographic evidence of sub-optimal anatomic construct led to multiple operative procedures. Psychological intervention relative to the persistent nausea and abdominal pain was ineffectual, although the supportive and consistent nature of the psychotherapy relationship was useful in overall patient stability and emotional well being. Complete reversal of the bypass did not effect improvement. Ultimately, the diagnosis of narcotic withdrawal prompted the institution of methadone treatment with complete cessation of the symptoms of nausea and pain. CONCLUSIONS: The diagnosis of narcotic withdrawal syndrome can be difficult in the postoperative bariatric patient. Psychological evaluation and support are essential elements of the program throughout the entire course of a patient's treatment experience. Laparoscopic techniques simplified the surgical care of this patient.

[Comparative study of glycosylated hemoglobin with oral glucose tolerance test in a selected population]. / Comparación de la hemoglobina glucosilada con la prueba de tolerancia oral a la glucosa en una población seleccionada.

A series of 405 consecutive oral glucose tolerance tests was analyzed in comparison with simultaneous glycosylated hemoglobin (HbA1c) measurements, in order to ascertain the possible utility of HbA1c as an alternative method for diagnosis and screening in populations suspected or at increased risk of presenting diabetes mellitus. The study group consisted of 158 male and 247 nonpregnant female patients aged 3 to 84 years (median 61.5 and 56 years, respectively) referred by their physicians for diagnostic purposes. Tolerance test was performed according to usual methods and HbA1c was measured with the 2000 DC immunoassay. Results showed a good correlation between HbA1c and fasting or 2 hour glucose levels. Using WHO diagnostic criteria, HbA1c maximal normal level of 5.4% showed a sensitivity of 0.96 in distinguishing between non-diabetics and those at increased risk, for screening purposes. With HbA1c levels of 6.0 or 6.3%, specificity for a correct diagnosis of diabetes was high (0.94 or 0.97) making this a suitable level for diagnostic confirmation. With the new ADA criteria for fasting plasma glucose, the results were similar. We suggest that HbA1c measurement with highly accurate methods might be considered a valid alternative for diagnosis and screening in populations suspected or at increased risk of presenting diabetes mellitus.

Erythrocyte alkaline phosphatase in patients with myotonic muscle disorders.

The allosteric behaviour of the p-nitrophenyl-phosphatase (E.C.3.1.3.1.) from membrane erythrocytes was investigated in the following multisystemic diseases: myotonic dystrophy, limb-girdle muscular dystrophy, Charcot-Marie-Tooth and juvenile spinal muscular atrophy; in myotonia congenita, which is not a multisystemic disease, and in healthy controls. The Hill coefficient in F inhibition in controls was different from that in multisystemic diseases patients but not from that in myotonia congenita patients. Changes in the cooperative type kinetics would suggest that the interaction membrane-enzyme in controls and in patients with neuromuscular disorders is only different for multisystemic diseases.

Recurrent mutations in the vasopressin-neurophysin II gene cause autosomal dominant neurohypophyseal diabetes insipidus.

We examined the nucleotide sequence of the arginine vasopressin-neurophysin II gene in three kindreds with autosomal dominant neurohypophyseal diabetes insipidus. Each of the three different mutations identified represents a recurrence of a mutation previously described to cause this disease. These mutations are all transitions (C1761-->T, G1859-->A, and G279-->A) that encode amino acid substitutions Pro24-->Leu, Gly57-->Ser (both in neurophysin II), and Ala-->Thr (in the last amino acid at the C-terminus of the signal peptide). The presence of these mutations in genomic DNA was confirmed by alterations in restriction endonuclease recognition sites. A linkage map of distal chromosome 20 was constructed. To examine the possibility that these apparent recurrent mutations arose independently rather than by an ancestral founder mutation, we analyzed family origins, two polymorphic markers on chromosome 20 in close proximity with this gene (the oxytocin/XbaI restriction fragment length polymorphism and the D20S57 polymorphic CA repeat microsatellite), and/or the occurrence of a de novo mutation in our three families and in four additional families previously reported. Our results suggest that one of our families may share an ancestral founder mutation with one previously reported family, but that in the remainder of the families with identical mutations, these mutations probably arose independently.

[Thyroid cancer: 20 years experience in a hospital]. / Cancer de tiroides: experiencia hospitalaria en 20 años.

A retrospective analysis of 117 cases of thyroid cancer diagnosed and/or treated at the Hospital Privado de Comunidad in Mar del Plata, Argentina, between 1974 and 1993 is presented and compared to previously published reports. Mean age at diagnosis was 54.3 years (range 17-88). Follow-up was 8.2 +/- 5.7 years. One third of patients had a previous history of thyroid disease. Histologic types had frequencies comparable to other series: 67.5% papillary, 15.4% follicular, 6% medullary, 3.4% Hürthle-cell and 6.8% undifferentiated. There was one case of lymphoma. Female to male ratio was 4.2:1 (95 females, 22 males). Higher age at diagnosis (over 63 years), higher tumor staging (TNM III or IV) and histologic type (undifferentiated, Hürthle and medullary) were important unfavorable prognostic factors for recurrence or death from the disease, but sex, type of surgery, postoperative radiation and hormonal therapy were not. Undifferentiated carcinomas (n = 8) had 100% mortality (survival 1-17 months after diagnosis, mean 5.5) and Hürthle-cell cancer had 75% mortality (survival 22-46 months, mean 33). Fine needle aspiration biopsy proved to be a useful diagnostic procedure, with an elevated positive predictive value (96%) and a moderate sensitivity (75%). A gradual decrease in surgical complications was observed, the last case of hypoparathyroidism occurring in 1986.

Maternal and neonatal variables in twins: an epidemiological approach.

Population studies on human twinning are scarce in Argentina. In order to analyze frequencies and certain maternal and neonatal variables related to twin births, we studied a series of 69.678 consecutive newborns with 500 g of weight and over, which occurred at a public hospital in the province Buenos Aires, during 14 years (1982-1995). The frequency of twin births (10 per 1000 deliveries) and sex ratio were similar to other studies reported in Caucasian population. Maternal age and order of gravity/parity were positively correlated with twinning rates, more markedly so in dissimilar sex-pairs. Stillbirths and neonatal deaths were more frequent in twins than in singletons, but less frequent when comparing groups of same weight. Congenital malformations were not found to be significantly more frequent in twins than in the total newborn population. However, their occurrence, predominantly in like-sexed pairs and the concordance for defect type in doubly affected same-sex pairs, suggests that monozygotic twinning carries an increased risk for malformation.

[Houssay's phenomenon in man]. / Fenómeno de Houssay en el hombre.

The phenomenon of clinical improvement of diabetes mellitus after occurrence of pituitary insufficiency has been reported occasionally in the medical literature, as a human counterpart of Houssay's experiment with hypophysectomized diabetic animals. We report the case of a 76-year-old woman who developed diabetes in 1928, at the age of 14, and was treated with low doses of insulin. At the age of 29, during the 7th month of her second pregnancy, she suddenly developed severe headaches and soon afterwards an intense polyuria which subsided under treatment with posterior pituitary extract. Her pregnancy followed to term but uterine stimulants had to be used at delivery because of lack of contractions. She was unable to nurse her baby and a permanent amenorrhea ensued. She continued using the posterior pituitary powder for several years, after which she discontinued it without adverse effects. The dose of insulin was decreased gradually until its replacement by chloropropamide in 1967 and glibenclamide in 1970. The present dose of glibenclamide is 2.5 mg daily, on which she has occasional mild hypoglycemic reactions. When the medication was discontinued for 5 days glycemia rose to 450 mg/dl but responded immediately to 2.5 mg of the drug with a mild hypoglycemia. She never required thyroid hormone therapy. Glucocorticoid substitution was instituted recently because of evidence of mild adrenocortical insufficiency. Basal hormone levels were normal for thyroxin, thyrotropin, FSH, LH, prolactin, hGH and cortisol; the responses to pituitary stimulation with TRH and LHRH were subnormal or nil. Cortisol stimulation with ACTH was normal. Insulin levels rose moderately after stimulation with glucagon, and with glibenclamide, with simultaneous marked decrease in glycemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Fenómeno de Houssay en el hombre / Houssay's phenomenon in the man

La mejoría clínica de la diabetes luego de sufrir una insuficiencia hipofisaria, equivalente humano del experimento de Houssay en animales diabéticos hipofisoprivos, ha sido descripta ocasionalmente en la literatura médica. Presentamos el caso de una mujer de 74 años de edad quien desarrolló diabetes a los 14 años, siendo tratada con dosis bajas de insulina. A los 29 años, en el 7§ mes de su 2§ embarazo, sufrió un episodio de cefalea intensa, seguido por poliuria severa que respondió al "polvo de hipófisis posterior". El embarazo continuó hasta el término, pero requirió medicación para producir contracciones; no pudo amamentar al bebé y entró en amenorrea permanente. El polvo de hipófisis fue suspendido años después, sin consecuencias. Las diabetes fue controlada con dosis decrecientes de insulina, hasta que a los 53 años fue reemplazada por clorpropamida, y a partir de los 56 años por glibenclamida. Actualmente, con dosis diaria de 2,5 mg de glibenclamida, suele tener hipoglucemias leves; al suprimiria, la glucemia se elevó a 450 mg/dl en 5 días. No requirió nunca medicación tiroidea, y sólo en los últimos 2 años, comenzó a requerir dosis diarias de 20 mg de hidrocortisona. Las determinaciones hormonales mostraron valores basales normales de T4, TSH, FSH, LH, PRL, STH y cortisol, con respuestas subnormales o nulas bajo estímulo (TRH, LHRH, ACTH, Arginina), excepto el cortisol cuya respuesta fue normal. La silla turca no mostró alteraciones radiográficas ni en la TAC. Los exámenes oftalmológico y neurológico, no indican evidencia de complicaciones diabéticas crónicas. La genealogía muestra diabetes en cuatro generaciones sucesivas. Concluimos que esta paciente, portadora de una diabetes de comienzo juvenil (posiblemente tipo MODY), sufrió una necrosis hipofisaria parcial a la edad de 29 años, y cumplió 60 años de su enfermedad sin evidencia de complicaciones y con marcada sensibilidad a la insulina, característica del fenómeno que Houssay describió en 1930

Fenómeno de Houssay en el hombre / Houssays phenomenon in the man

La mejoría clínica de la diabetes luego de sufrir una insuficiencia hipofisaria, equivalente humano del experimento de Houssay en animales diabéticos hipofisoprivos, ha sido descripta ocasionalmente en la literatura médica. Presentamos el caso de una mujer de 74 años de edad quien desarrolló diabetes a los 14 años, siendo tratada con dosis bajas de insulina. A los 29 años, en el 7º mes de su 2º embarazo, sufrió un episodio de cefalea intensa, seguido por poliuria severa que respondió al "polvo de hipófisis posterior". El embarazo continuó hasta el término, pero requirió medicación para producir contracciones; no pudo amamentar al bebé y entró en amenorrea permanente. El polvo de hipófisis fue suspendido años después, sin consecuencias. Las diabetes fue controlada con dosis decrecientes de insulina, hasta que a los 53 años fue reemplazada por clorpropamida, y a partir de los 56 años por glibenclamida. Actualmente, con dosis diaria de 2,5 mg de glibenclamida, suele tener hipoglucemias leves; al suprimiria, la glucemia se elevó a 450 mg/dl en 5 días. No requirió nunca medicación tiroidea, y sólo en los últimos 2 años, comenzó a requerir dosis diarias de 20 mg de hidrocortisona. Las determinaciones hormonales mostraron valores basales normales de T4, TSH, FSH, LH, PRL, STH y cortisol, con respuestas subnormales o nulas bajo estímulo (TRH, LHRH, ACTH, Arginina), excepto el cortisol cuya respuesta fue normal. La silla turca no mostró alteraciones radiográficas ni en la TAC. Los exámenes oftalmológico y neurológico, no indican evidencia de complicaciones diabéticas crónicas. La genealogía muestra diabetes en cuatro generaciones sucesivas. Concluimos que esta paciente, portadora de una diabetes de comienzo juvenil (posiblemente tipo MODY), sufrió una necrosis hipofisaria parcial a la edad de 29 años, y cumplió 60 años de su enfermedad sin evidencia de complicaciones y con marcada sensibilidad a la insulina, característica del fenómeno que Houssay describió en 1930 (AU)

Fenómeno de Houssay en el hombre. / [Houssays phenomenon in man]

The phenomenon of clinical improvement of diabetes mellitus after occurrence of pituitary insufficiency has been reported occasionally in the medical literature, as a human counterpart of Houssays experiment with hypophysectomized diabetic animals. We report the case of a 76-year-old woman who developed diabetes in 1928, at the age of 14, and was treated with low doses of insulin. At the age of 29, during the 7th month of her second pregnancy, she suddenly developed severe headaches and soon afterwards an intense polyuria which subsided under treatment with posterior pituitary extract. Her pregnancy followed to term but uterine stimulants had to be used at delivery because of lack of contractions. She was unable to nurse her baby and a permanent amenorrhea ensued. She continued using the posterior pituitary powder for several years, after which she discontinued it without adverse effects. The dose of insulin was decreased gradually until its replacement by chloropropamide in 1967 and glibenclamide in 1970. The present dose of glibenclamide is 2.5 mg daily, on which she has occasional mild hypoglycemic reactions. When the medication was discontinued for 5 days glycemia rose to 450 mg/dl but responded immediately to 2.5 mg of the drug with a mild hypoglycemia. She never required thyroid hormone therapy. Glucocorticoid substitution was instituted recently because of evidence of mild adrenocortical insufficiency. Basal hormone levels were normal for thyroxin, thyrotropin, FSH, LH, prolactin, hGH and cortisol; the responses to pituitary stimulation with TRH and LHRH were subnormal or nil. Cortisol stimulation with ACTH was normal. Insulin levels rose moderately after stimulation with glucagon, and with glibenclamide, with simultaneous marked decrease in glycemia.(ABSTRACT TRUNCATED AT 250 WORDS)

The clinical spectrum of alpha-L-iduronidase deficiency.

We present five patients with alpha-L-iduronidase deficiency who do not have the typical Hurler or Scheie phenotypes; they are compared to 28 similarly atypical cases from the literature. Phenotypic differences are pointed out and intrafamilial similarities stressed. Among the various possible explanations for this situation, the existence of genetic compounds seems acceptable for some of the cases, but others seem to be caused by different mutations. The elucidation of these alternative possibilities from recent biochemical research is discussed.

Weyers acrodental dysostosis in a family.

A four generation family with postaxial polydactyly of hands and feet and dental anomalies is reported. Lower and upper incisors were abnormal in shape and number. Additional findings were prominent ear anthelices, hypoplastic and dysplastic nails and mild shortness of stature. Inheritance was dominant with variable expression. It is proposed that the family presents the syndrome of acrofacial dysostosis described by Weyers in 1952. To differentiate it from other acrofacial dysostoses, we suggest naming the condition acrodental dysostosis.

Heterogeneity of metatropic dysplasia.

Metatropic dysplasia is a neonatally manifest entity that is characterized clinically by a rapidly progressing kyphoscoliosis leading to severe shortening of the originally long trunk ("metatropism"). Major radiographic features include flattening and defective ossification of the vertebral bodies, a narrow thorax and a marked hypoplasia of the basilar portions of the ilia with crescent-shaped iliac crests. There is some evidence of genetic heterogeneity. From five personal observations and from a review of the literature we conclude that metatropic dysplasia comprises at least three genetic entities: (1) a nonlethal type with autosomal recessive transmission; (2) a nonlethal dominant type and (3) a lethal type with death before or shortly after birth and possibly autosomal recessive inheritance. A careful clinical and radiologic analysis permits their classification and differentiation from similar, not yet well-delineated disorders. Attention is also drawn to the possibility of atlanto-axial instability in metatropic dysplasia which may cause severe neurologic defects.