Biological Significance of Anti-GH Antibodies in Children Treated with rhGH

Background: The occurrence of antidrug antibodies is common in children treated with recombinant human growth hormone (rhGH). However, their clinical significance is unclear. Objective: This study aimed to examine the clinical significance of anti-GH antibodies by analyzing the phenotype of patients who tested positive in relation to the quantity of anti-GH antibodies. Method: In this laboratory-based retrospective study encompassing a time span of 6 years, all positive samples were identified, and senders were contacted. Anti-GH antibodies were measured using a radioprecipitation assay; positive samples underwent a confirmatory assay. Results: Out of a total of 104 samples from 66 patients, positive test results were found in 28 samples from 13 patients. Clinical data were available from all but one. The group with positive test results comprised 6 patients with a normal response to GH provocative tests (group A) and 6 with an insufficient response or with isolated GH deficiency (IGHD) type 1A (group B). Diagnoses in group A were neurosecretory dysfunction, bioinactive GH syndrome and constitutional delay of growth and puberty. Diagnoses in group B were IGHD type 1A, septo-optic dysplasia, and cerebral midline defect with multiple pituitary hormone deficiency. Insufficient growth response to rhGH was absent except in one sibling pair with IGHD type 1A and a patient with cerebral midline defect. These patients had the highest concentrations of anti-GH antibodies. Conclusions: The biological significance of anti-GH antibodies seems to be limited to patients with high concentrations of anti-GH antibodies. For all other patients, we recommend a careful “wait and see” strategy and monitoring antibody titers.

A Neonate with an Unusual Midline Defect and Cardiovascular Anomaly

We present a female neonate with a sternal cleft (SC) and additional aortic aneurysm who presented with respiratory failure. Stabilization of the SC was achieved by using the xyphoid process as an autologous graft bridging the upper part of the SC. We conclude that a step-wise correction of the SC with the use of an autologous graft may improve respiratory function, and should be considered when complete surgical correction is not feasible.

Schistosomus reflexus in a Dog and a Cat

Background: Schistosomus reflexus (SR) is a congenital anomaly, that the animal presents exposed abdominal and thoracic organs. The alterations includes limbs malformation, abnormal spinal column, liver and diaphragm hypoplasia, genitourinary and gastrointestinal changes, on this way, the fetus can not survive. The aim of this study was to report the first SR case in dog and cat in Brazil, and describe anatomical, pathological and radiographic changes.Case: The puppy had ventral midline defect in the abdominal and thoracic wall extending from the sternum to the pubis with viscera exposition, including the heart, lung, liver, stomach, intestine, spleen, kidneys, moreover right army was abnormal, however no spinal column was present. It was performed radiography to evaluate bone abnormalities, and was diagnosed agenesis of carpal, metacarpal and phalange bones. The kitten had ventral midline defect measuring 4 x 2 cm, in the abdominal wall and exposition of liver, stomach, intestine and spleen, as well as tibiotarsal joint arthrogryposis on both lags. It was performed fetus radiography and no spinal column change was diagnosed.Discussion: Schistosomus reflexus is a congenital abnormality common in ruminants. Although it was few reported in dogs and cats. The two cases in this study are the firsts reposts in Brazil in these especies. In our case, the dog had exposition of abdominal and thoracic organs and died one hour after born, similar to this, another author reported a case where the thoracic and abdominal evisceration and the puppy died ten minutes after born. However, in another case with abdominal evisceration only, the puppy survived for eight days. Probably it happened because respiratore function was not compromised. The kitten had abdominal opening only, in turn, other authors reported a case whose abdominal viscera exposition was by pelvic fissure, and another case, what presented thoracic and abdominal evisceration, like our dog case. Some authors affirm that this defect causes fetal dystocia on ruminants, because of the vertebral column abnormality, what promote passage difficult at born moment. On the other hand, dogs did not present these kind of changes and dystocia was not related to this pathology on cases in dogs and cats, however most of these authors performed cesarean because the female did not get normal born. As well as, on two cases in kitten were reported vertebral column abnormalities, what can be related to dystocia. Even no animal of our study present any vertebral column abnormality, other bone changes were found, like carpal, metacarpal and phalange agenesis on army of the dog and tibiotarsal joint arthrogryposis on both lags of the cat fetus. The others report in dogs did not found vertebral column or bone changes, but in cats it was observed vertebral column abnormalities and other changes, like arthrogryposis on four limbs, skull flattening, mandibular brachignathism, reduced ossification of the cranial vault bones, palatoschisis and unfused eyelids. SR etiology is not clearly known, it may be related with genetic, mechanic, endocrine, metabolic, nutrition and infectious factors. The moment of embryonic development, where these factors influence the changes is related with abnormality grade. Although, Chromosomic abnormalities, like chromatid and chromosome breaks, non-homologous pairing of chromosomes was shown. In this study the bitch did not present any metabolic, endocrine or nutritional change, on other hand the queen was submitted to exogenous hormonal treatment to avoid pregnancy, what may be related to SR development. SR is a rare abnormality in dog and cat, fetus radiographic, anatomical and pathological findings confirmed to being this congenital defect, and this are the first reports in Brazil in these species.

A case of midline defect of posterior arch of atlas in a cadaver

During a routine osteology class for undergraduate students in Government Medical College, Kozhikode, Kerala, a median defect of posterior arch was found in the atlas of a cadaver. It was not a fracture as the edges were regular, round and smooth.There was no associated anomalies. These midline defects of posterior arch of atlas are sub divided both morphologically (types A-E) and clinically (sub groups 1-5) depending on the extent of absence of posterior arch and presence or absence of the posterior tubercle.The author presents a rare anomaly of a posterior arch partial aplasia which could be congenital. Since major neurologic deficits can be produced by a minor trauma, it is crucial to recognize this anomaly.