Cryptophthalmos.

Cryptophthalmos, a very rare congenital anomaly of the eye, is characterised by skin passing continuously from the forehead to the cheek over a malformed eye. It may be isolated or more commonly as a part of Fraser syndrome. Cryptophthalmos is classified into three types: complete, incomplete and abortive. Surgical reconstruction is the modality of treatment available. We present a case of bilateral, complete, isolated cryptophthalmos, who has undergone stage 1 of reconstructive surgery.

Familial congenital valvar pulmonary stenosis: autosomal dominant inheritance.

A mother and three of her four children, one girl and two boys, who had valvar pulmonary stenosis are described. One child had an associated septum secundum atrial septal defect, and another had an associated ventricular septal defect. An autosomal dominant mode of inheritance is likely for the valvar pulmonary stenosis. The association of additional cardiac lesions in two of the three siblings raises the possibility of closely associated genetic loci.

Reactogenecity of indigenously produced measles vaccine.

OBJECTIVE: To assess the safety and reactogenecity of indigenously produced measles vaccine derived from EZ strain. DESIGN: A longitudinal clinical follow up after vaccination. SETTING: Hospital based and home follow up, as required. SUBJECTS: 12,470 children, 9 to 15 months old, immunized with measles vaccine of EZ strain, in accordance with the National Immunization Schedule, at five centers. METHODS: A clinical follow up of children at 1 day, 1 week, 2 weeks, 3 weeks and 6 weeks after measles vaccination. A detailed clinical neurological examination in children showing side effects. RESULTS: Mild side effects were documented in 31%. Of these, 90% were seen in the first two weeks, out of which two thirds were seen during the first week. Commonest side effects were coryza (10%), fever (9.8%), cough (3.2%) and diarrhea (3.2%). Convulsions, with no later sequelae were documented in 2 cases only. CONCLUSIONS: Measles vaccine manufactured in India, using EZ strain is a safe vaccine. It has a level of reactogenecity including neurological aspects, lower than that reported in India with the Schwarz strain vaccine.

Immunogenicity and reactogenicity of indigenously produced MMR vaccine.

OBJECTIVES: To study immunogenicity and reactogenicity of indigenously produced MMR vaccine and to assess the booster effect of MMR immunization on measles seroconversion. DESIGN: A longitudinal follow up. SETTING: Hospital based and home follow up, as required. SUBJECTS: 89 children already immunized for measles, between 15 to 24 months of age for immunogenic evaluation and 866 subjects for the reactogenic component. METHODS: Prevaccination and postvaccination samples collected one and four weeks after vaccination were studied by ELISA for IgG and IgM antibodies against the three diseases. A clinical follow up of immunized children was done at 3 days, 7 days, 6 weeks and 6 months after immunization. RESULTS: IgG positivity 4 weeks after immunization rose from 75% to 100% for measles, from 12% to 92% for mumps, and from 13% to 99% for rubella. Only mild side effects including pain and swelling in 37 (4.3%) cases, mild fever in 51 (5.9%) cases, cough in 40 (4.6%) cases and a transient rash in 7 (0.8%) cases were observed. CONCLUSIONS: The indigenously manufactured MMR vaccine has an excellent immunogenicity and low reactogenicity with a booster effect for measles seroconversion in children already immunized for this disease.

Klippel-Trenaunay-Weber syndrome with hydrocephalus: an unusual association.

Macrocephaly is an associated finding with Klippel-Trenaunay-Weber syndrome, the cause of which remains to be determined. Association of this syndrome with hydrocephalus due to aqueductal stenosis has not been reported previously in world literature and is presented in this clinical brief.

Neonatal mydriasis due to effects of atropine used for maternal Tik-20 poisoning.

A neonate was born to a mother who had consumed an organophosphorus(OPC) compound with suicidal intent. The mother was administered atropine and this caused mydriasis in the neonate without any other pharmacological effects. There was no evidence of placental dysfunction. There are no case reports of OPC consumed in pregnancy and its effect on neonates or of effects of massive doses of atropine in the mother and its effects on the fetus or the newborn.

Spondylocostal dysostosis.

Spondylocostal dysostosis is a rare condition characterized by short stature due to a short trunk, multiple morphological abnormalities of the vertebrae and ribs due to malsegmentation of the axial skeleton. Radiological features include reduced number of vertebrae and ribs, hemivertebrae, fused or sagitally cleft vertebrae or multiple rib fusions. Three distinct clinical entities are described. We report nine cases of this syndrome, seven were infants and presented with an abnormal shape of the thorax. Two of them had a meningocoele, and one succumbed to the CNS anomalies on the third day of life. The other two cases were two and eight years of age. Cardiac lesion was detected in one case and renal malformations in three cases. Reduction in the rib number was present in all cases, and rib fusion in seven cases. Thoracic vertebral dysegmentation was noted in all, lumbar in three and cervical in one case. This is the first large series from India. No clear single etiology was established.

The foetal immune response to maternal tetanus toxoid immunization.

Paired maternal and cord blood samples were collected at delivery from 150 women who received varying doses of tetanus toxoid during pregnancy. Tetanus specific IgM and IgG antibodies were measured in them by standard ELISA with a sensitivity for IgM of 0.001 mg/ml, and for IgG of 0.0003 IU/ml. In 22 infants an additional estimation of tetanus antibody was made 1 month after birth. The presence of specific IgM in 78% of cord samples established an active foetal immune response. The titre did not alter significantly with the number of TT doses given to the mother. Foetal IgM rose in 60% of cases at one month of age compared to cord blood levels. At this time IgG levels were uniformly diminished in accord with a maternally derived passively transferred antibody. No switch of foetal IgM to IgG production was evident. The foetal immune response thus did not confer active protection against tetanus.

Micromethod for PMN function studies in neonates.

Polymorphonuclear neutrophils play an important role in host defense mechanism. To assess PMN function in neonate a micromethod using only a few drops of blood was standardised. PMN adherent to coverslip were incubated with bacteria and using a differential staining with Acridine Orange (AO) phagocytic and bactericidal capacity were estimated.

In vitro effect of intravenous immunoglobulin on serum opsonic activity in normal & intrauterine growth retarded neonates.

In vitro effect of intravenous immunoglobulin (IVIG), Intraglobin F, on serum opsonic activity against Staphylococcus aureus was studied in 26 full term normal healthy neonates and 18 intrauterine growth retarded (IUGR) neonates by the polymorphonuclear leucocyte overlay method (requiring only a few drops of blood). Cord IgG and IgM levels were determined by single radial immunodiffusion. Serum opsonic activity against Staph. aureus was significantly lower in the IUGR neonates (49.1 +/- 0.89), as compared to that in normal neonates (61.96 +/- 0.73; P less than 0.001). Immunoglobulin supplementation in vitro at a concentration of 5 g/dl significantly enhanced the opsonic activity of IUGR neonate sera. Cord IgG levels of IUGR neonates were significantly lower (P less than 0.01) than IgG levels of normal neonates. No significant difference was observed in cord IgM levels between the normal and IUGR neonates.

Neonatal osteomyelitis and septic arthritis.

The records of seventeen neonates (13 males and 4 females) with osteomyelitis and septic arthritis, who were admitted over a 3 year period, were reviewed. Neither prematurity nor low birth weight seemed to be a predisposing factor. The majority of the neonates (88.2%) had some antecedent illness or were subjected to certain procedures in the neonatal period. Paucity of movement of limb, fever and local swelling of the joint were the most frequent presenting symptoms. Deep soft tissue swelling was the frequently noted radiographic change (58.8%). Bone scintigraphy was abnormal in 7 out of 11 cases. Of the 21 sites involved, femur and humerus accounted for 76.1% of cases. Four of seventeen neonates had multiple bone involvement. Arthritis was present in fifteen neonates. Of the organisms isolated, Staphylococcus aureus was the commonest.