A Rare Case of Congenital Nephrogenic Diabetes Insipidus Associated with Aquaporin 2 Gene Mutation and Subsequent Acute Lymphoblastic Leukemia: Impact of Steroids on Kidney Function.

BACKGROUND Nephrogenic diabetes insipidus (NDI) is a rare renal disorder that can be congenital, and is caused by mutations in either aquaporin 2 or arginine vasopressin receptor 2, or it can be secondary to kidney disease or electrolyte imbalance. The clinical signs of NDI include polyuria, compensatory polydipsia, hypernatremic dehydration, and growth retardation without prompt treatment. In this report, we present the case of a patient with congenital NDI who was later diagnosed with acute lymphoblastic leukemia (ALL). With dexamethasone treatment, he had uncontrolled polyuria and polydipsia. Our aim was to concentrate on the impact of steroids on the kidneys. CASE REPORT Our patient presented at the age of 9 months with signs of severe dehydration that were associated with polyuria. His laboratory examinations revealed hypernatremia and decreased urine osmolality. He was diagnosed with NDI and his exome sequence revealed a homozygous mutation at the nucleotide position AQP2 NM_000486.6: c.374C>T (p.Thr125Met). He was treated with hydrochlorothiazide and amiloride. Then, at age 19 months, he presented with gastroenteritis and a complete blood count (CBC) showed high white blood cell count and blast cells. He was diagnosed with (ALL) and began receiving chemotherapy, during which again developed polydipsia and polyuria, which could not be controlled with an increased dosage of hydrochlorothiazide. CONCLUSIONS We report a rare case of NDI caused by a missense mutation in the aquaporin 2 gene. One year later, the child developed ALL, and treatment with dexamethasone led to an uncompensated state of polydipsia and polyuria.

An Unusual Complication of Measles Infection in a Pediatric Patient: A Case Report of Measles-Induced Hemophagocytic Lymphohistiocytosis (HLH) From Jordan.

Measles is a highly contagious infection that leads to many serious complications. Despite the significant global effort to eradicate it, it still represents a major threat due to suboptimal vaccination coverage, especially after the coronavirus disease 2019 (COVID-19) pandemic that affected all routine childhood vaccinations. One of its fatal complications, which has been reported a few times in the literature, is hemophagocytic lymphohistiocytosis (HLH). We discuss a case of a 14-month-old unvaccinated female patient who developed measles-induced HLH and was treated with intravenous immunoglobulins (IVIG) and steroids; unfortunately, she developed multiorgan failure and passed away before chemotherapy could be initiated.

Indications and Clinical Profile of Neonatal Admissions: A Cross-Sectional Descriptive Analysis from a Single Academic Center in Jordan.

PURPOSE: To review the indications and clinical profile of neonatal admissions at King Abdullah University Hospital in Jordan. MATERIALS AND METHODS: We conducted a cross-sectional review of all neonates admitted to the neonatal intensive care unit between September 2016 and September 2018. Collected data include demographic characteristics, indications for admission, morbidities and mortality, and discharge outcomes. Findings were reported among term and preterm infants. RESULTS: A total of 1444 infants were admitted during the study period of whom 1332 (92.2%) were inborn and 612 (42.4%) were term neonates. Of the 832 preterm infants, 545 were late preterm (34-36 6/7 gestation) and 125 had very low birth weight (˂ 1500 grams); 925 (64%) were born by cesarean section. Respiratory failure of the newborn (41.2%) and prematurity (33.3%) were the main indications for admission among the whole cohort. Maternal prolonged premature rupture of membranes (PROM) was observed in nearly half the admissions of term infants. Hypoxic ischemic encephalopathy (3.2% vs 0.7%, p 0.01) and congenital anomalies (5% vs 1.2%, p 0.03) were more common in term infants. The rate of bronchopulmonary dysplasia was 39% among <28-week and 28% among <32-week premature infants. Sepsis was encountered in 59 infants. The overall mortality rate was 3.8%. Prematurity was the main predisposing factor for mortality (Adjusted OR: 9.9, 95% CI: 3.5, 27.6). CONCLUSION: The majority of neonatal admissions at our institution are term and late preterm infants delivered by cesarean section. Prematurity, respiratory failure of the newborn, and suspected sepsis due to maternal PROM are the leading causes of admission. The mortality rate is within WHO target to achieve Sustainable Development Goal 3. Population-based studies are needed to make better conclusions that represent the whole Jordanian population. A revisit for the indications of cesarean deliveries may help to improve the neonatal outcomes.