[Beta-cell, thyroid and celiac autoimmunity in children with type 1 diabetes]. / Autoinmunidad pancreática, tiroidea y relacionada con la celiaquía en niños con diabetes mellitus tipo 1.

OBJECTIVE: The aim of this study was to evaluate the presence of beta-cell, thyroid and coeliac autoimmunity in children with recent-onset type 1 diabetes. METHODS: We studied all children with newly diagnosed type 1 diabetes. Data were analyzed from 63 children aged 2-14 years who were treated in the Department of Pediatrics of the Virgen de las Nieves University Hospital in Granada (Spain) from 1998-2002. Antibodies to glutamic acid decarboxylase-65 (GADA), anti-insulin (AIA), thyroperoxidase (anti-TPO), thyroglobulin (anti-TG), thyroid-stimulating immunoglobulins (TSI) and endomysial antibodies (EmA-IgA) were measured and documented. A total of 55.5 % of these patients were girls and the mean age was 7.9 +/- 3.2 years. RESULTS: The prevalence rates were: GADA 65.1 %; AIA 68.3 %; anti-TPO 11.1 %; anti-TG 9.5 %; TSI 4.8 % and EmA-Ig A 3.1 %. Children with thyroid antibodies (anti-TPO1) were significantly older and developed diabetes later in life (P < 0.05) than those without antibodies. Thyroid-stimulating hormone levels, goiter and thyroid dysfunction were higher in children who were anti-TPO1 than in diabetic children without thyroid autoimmunity. CONCLUSIONS: Children with newly diagnosed type 1 diabetes show a high prevalence of thyroid and coeliac autoimmunity. In cases of positivity, additional work-up and intervention are advocated. These measures can prevent the well-known complications of diabetes and improve its clinical course.

[Recurrent bowel intussusception and celiac disease]. / Invaginación intestinal recidivante y enfermedad celíaca.

We report a 1-year-old girl with acute abdominal pain. Clinical examination revealed distended abdomen with increased intestinal peristalsis without other pathologic signs. Ultrasound of the abdomen showed a small bowel intussusception. Seven days later, after a new episode of sudden acute abdominal pain, ultrasound revealed a new bowel intussusception. Ig A and Ig G antigliadin-antibodies were elevated and intestinal biopsy revealed total villous atrophy. After being placed on a gluten-free diet the girl was free of colicky complaints and on repeated ultrasound there was no sign of intussusception. With recurrent intussusception, celiac disease should be considered as a cause.

[Infrequent manifestations of Henoch-Schonlein purpura]. / Manifestaciones infrecuentes en la púrpura de Schönlein-Henoch.

We review the cases of 148 children with Henoch-Schönlein purpura diagnosed in a period of seventeen years (from 1975 to 1992). Thirteen cases (8.7%) of unusual clinical manifestations were found: testicular affection in six cases (7.6% of the males); IgA nephropathy in four (2.7%); exudative enteropathy in two (1.3%); duodenal syndrome in two; previous abdominal manifestation which led to surgical procedures in one (0.7%) and another case with detachment of the retina. These clinical findings are analyzed and the possibility of unnecessary therapeutic acts is discussed.

[Objective evaluation of a pediatric intensive care unit without continuous attendance (I)]. / Valoración objetiva de una UCI pediátrica de referencia sin atención continuada. (I).

Eight-hundred and nine consecutive admissions have been reexamined by means of analysis of record cards made out at the moment of discharge from the ICU, in which, among other data, the TISS and PSI maximum for the first six hours from admission are included. The results obtained are the following: 50% of the admissions were classified as medium care (PSI < 5, TISS < 20), 28% were grade IV (PSI > 12, TISS > 40), and half were of grade III with a PSI of 9.38 and a TISS of 20.29. The overall mortality was 8.9%. However, the mortality for grades III+IV was 13.4% and gave a good correlation with the PSI, but not with the TISS. The PSI/TISS ratio was 0.45 and the predicted mortality rate according to the PSI was 6.1% for grade III and greater than 12% for grade IV. We conclude the following: (1) Of the illness, 28% were grade IV. (2) We observed a deficient PSI/TISS ratio. (3) The evaluation has to be done by objective means in which the capability of carrying out different methodologies has not been taken into account.

[Parathyroid insufficiency syndrome. Two new cases: type Ia pseudohypoparathyroidism and pseudo-idiopathic hypoparathyroidism]. / Síndrome de insuficiencia paratiroidea. Dos nuevos casos: pseudohipoparatiroidismo tipo Ia e hipoparatiroidismo pseudoidiopático.

Two new cases with parathyroid insufficiency syndrome are described. The first one is a seven year and 6 months old male with Pseudohypoparathyroidism Type Ia, who has presented: hypocalcemia, hyperphoforemia, increased PTH and TSH, prolactin decreased and Albright hereditary osteodystrophy phenotype. The second one in a six year and four months old female with Pseudo-idiopathic hypoparathyroidism who has presented: hypocalcemia, hyperphosforemia, increased PTH, without any hormonal disturbances nor dysmorphias. Ellsworth-Howard test with 50 micrograms of 1-34 hPTH is made in both, remarking the different renal response about phosphaturia and plasmatic, urinary and nephrogenic cyclic AMP. The treatment is performed in both with calcitriol and oral calcium (L-Thyroxine is associated for the first patient). Biochemical disturbances are normalized. Other parathyroid insufficiency syndrome are revised.

[Congenital generalized fibromatosis. Presentation of a case and review of the literature]. / Fibromatosis congénita generalizada. Presentación de un caso y revisión de la literatura.

We report a case of congenital generalized fibromatosis in a newborn who presented with a solitary subcutaneous tumour, developing subsequently a widespread generalization (soft tissues, bone and lung). The tumours were found to be fibromatosis pathological on study. A review of the literature related to the clinic aspects and to the prognosis is made.

[Infantile nephrosis. Microcystic renal disease (author's transl)]. / Síndrome nefrótico del lactante. Enfermedad microquística del riñón

Two siblings, male and female with nephrotic syndrome of neonatal onset are reported. Both parents were healthy as well as another 5 year-old brother. Both died under two months. Their clinical, biochemical and immunological data are described, and the more common causes of N.S. (i.e. syphilis, toxoplasmosis, tuberculosis) ruled out. Pathology was available only from one of the cases, and the lesions were comparable to those described by finnish authors as corresponding to "congenital" N.S. This report is an additional evidence of the presence of this entity out of Finland.