A Rare and Unusual Case of Hypernatremic Dehydration in a Newborn Presenting With Adrenal Haemorrhage and Leading to Acute Kidney Injury.

Adrenal haemorrhage, although a rare entity in the neonatal period, is a known complication of birth asphyxia. Adrenal haemorrhage progresses differently depending on the type and extent of the glands involved. Adrenal haemorrhage can cause persistent jaundice, fever, dehydration, scrotal swelling, abdominal wall discolouration, septicemia, and a shock-like state. Here, we report the case of a four-day-old male infant who presented with jaundice, poor feeding, and hypernatremic dehydration. The patient developed acute kidney injury and, eventually, renal failure due to adrenal haemorrhage. He had an abdominal lump with deranged renal parameters along with hyperbilirubinemia. Abdominal ultrasonography and contrast computed tomography scan showed left suprarenal enlargement with evidence of adrenal haemorrhage. The patient was managed well with ventilatory support and peritoneal dialysis and discharged successfully. A subsequent follow-up showed complete resolution of the adrenal haemorrhage. Single ultrasonography is a good modality for diagnosis but not sufficient, so serial ultrasonography at subsequent follow-up is a must.

Femoral Artery Thrombosis: Rare Sequelae of a Common Entity.

Neonatal hypernatremic dehydration (NHD) is a common complication in breastfed neonates which if not recognized early can lead to life-threatening complications. Only a few cases of NHD leading to peripheral gangrene have been reported in the literature. We report a case of a 14-day-old neonate with complaints of dyspnoea, poor oral intake, and gangrenous changes in the left leg. There was a 28% weight loss since birth due to inadequate feeding. The baby was diagnosed with severe hypernatremic dehydration with Kidney Disease Improving Global Outcomes (KDIGO) stage 4 acute kidney injury requiring peritoneal dialysis. Ultrasonography of the left lower extremity revealed a distal femoral artery thrombus leading to dry gangrene requiring amputation. There were neurological signs like altered sensorium and drug-resistant seizures which were suspicious for intracranial pathology like cerebral venous sinus thrombosis. Prevention and early diagnosis of NHD are essential to prevent the occurrences of such grave complications. It can be easily achieved by improving the vigilance regarding the adequacy of feeds subjectively by the mother if the baby is at home and objectively by physicians in the hospital setting. These simple interventions have the potential to prevent readmissions due to not only simple feeding complications but grave complications as mentioned above as well and save precious lives.

Concurrent diabetic ketoacidosis and pancreatitis in Paediatric acute lymphoblastic leukemia receiving L-asparaginase.

BACKGROUND: Although hyperglycemia and pancreatitis are known side effects of L-asparaginase, both contributing to the development of diabetic ketoacidosis (DKA) is unfamiliar in literature. CASE PRESENTATION: We report a case of an adolescent girl, recently diagnosed with ALL, who presented with pain in abdomen and breathing difficulty following chemotherapy with L-asparaginase. On subsequent evaluation, she was found to have high anion gap metabolic acidosis, hyperglycemia and ketonuria. Ultrasonogram showed bulky pancreas. DKA was managed with fluid correction and insulin infusion. Pancreatitis was managed conservatively. She recovered completely with resolution of symptoms and without any major adverse events despite having such severe complications. CONCLUSION: We conclude that the combination of DKA and pancreatitis is a rare occurrence with significant morbidity and mortality. We recommend a close monitoring of blood glucose levels for hyperglycemia as well as a high index of clinical suspicion for pancreatitis in patients with ALL receiving L-asparaginase.

Curse of Fluorosis.

Fluoride was identified to have caries preventive properties and was widely used for fluoridation of water since 1940, especially in developed countries. After this there was sudden increase in the use of fluorides in food items and in oral medicinal products like toothpastes and mouth washes. Inadvertent use of above has lead to increase in fluorosis as a public health problem. In many places high fluorides are naturally present in earth crust leading to high water fluoride content increasing the risk of fluorosis. Maintaining a fine balance of fluorides in the body is mandatory for exploiting its advantages. World Health Organization (WHO) has fixed permissible limit of fluorides in water to 1.5 mg/L as a preventive step to contain fluorosis. Fluorosis has three clinical components: Dental, Skeletal and Non-Skeletal Fluorosis. It occurs with increasing level of fluorides in the body. Acute toxicity due to fluorides is also known and occurs as a result of sudden exposure to high levels of fluorides, usually by ingestion. Once fluorosis occurs it is irreversible without any cure. Only symptomatic and supportive management is possible. Hence prevention is the mainstay of management. Prevention is by using alternative sources of water or its de-fluoridation. National Program for Prevention and Control of Fluorosis (NPPCF) was launched in 2008-9 to identify areas with high fluoride content of water, manage the water bodies, screen schools and community for fluorosis and comprehensive management of cases. Improving quality of drinking water as per standards and improving nutritional status of children are also important components of prevention of fluorosis.

Semilobarholoprosencephaly - A Dreading Congenital Anomaly.

Holoprosencephaly (HPE) is a group of structural abnormalities of brain that is an important cause of childhood mortality and morbidity. They usually occur due to impaired midline cleavage of embryonic forebrain i.e., failure of differentiation of the prosencephalon into the telecephalon and diencephalon. De Myer classified this anomaly ranging from alobar to semilobar and lobar type. It can be associated with microcephaly and midline facial anomalies. We present a case of semilobar holoprosencephaly with corpus callosal agenesis.

Suboptimal Performance in Cleft Lip/Palate Children- Who is Responsible?

Information in this article is from an observational study comparing intelligence in children with cleft lip and palate with normal children. Both groups performed "draw a man test", the investigator noted the attitude and behaviour of children and their parents. The study shows low, but normal intelligence quotient in children with oral defects as compared to normal. The probable reason for sub-normal performance appeared to be overprotective attitude of parents and poor self esteem of children with oral defects.

Is MRI Necessary for Skeletal Evaluation in Sickle Cell Disease.

BACKGROUND: More than 50% of the world's cases of sickle cell anaemia are in India with an estimated population of 1.27 billion as against estimated world's population of 7.24 billion. AIM: MRI of 103 patients of sickle cell disease were evaluated to assess the skeletal changes in proven cases of sickle cell disease and to find the incidence of bony infarcts in such patients. The conversion of red marrow to yellow marrow in these patients were also studied. MATERIALS AND METHODS: Sickle cell patients with musculoskeletal pain as well as asymptomatic sickle cell patients were evaluated by MRI. The standard sequences used wereT1WI, T2WI, STIR, T1WI + Gd Contrast. RESULTS: Persistent Red marrow was seen in axial and appendicular skeleton (62 cases). Extramedullary haematopoiesis was found in 4 cases, avascular necrosis of femur head (32 cases) and bone infarcts (46 cases) were also observed in our study. Osteomyelitis, septic arthritis and tubercular infections were associated with sickle cell disease in our study. CONCLUSION: MRI is very sensitive in detecting early stages of avascular necrosis, red marrow persistence, extramedullary haematopoiesis, changes of arthritis, infections and joint effusion.