Ermuntert euch, Aria, No. 5 from Cantata No. 176: Es ist ein trotzig und verzagt Ding (Alto Part)

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Major clinical differences between these two types of rickets include short stature and bowing, particularly of the lower extremities. Bowing is more frequently present in hypophosphatemic rickets. Insufficiency fractures and looser zones are also more commonly seen in hypophosphatemic rickets than in the vitamin D-deficient type of rickets. Frontal tibia and fibula radiograph shows diffuse osteopenia, metaphyseal splaying, fraying, and cupping of the distal femur, tibia arrow , and fibula.

Most cases of rickets respond well to vitamin D therapy and, if necessary, calcium supplementation. Affected pediatric patients also often present with renal stones. In comparison to adults with PHPT, affected pediatric patients present with a higher frequency of symptoms and more end organ damage. Neonatal HPT has an autosomal recessive inheritance or may be due to maternal hypothyroidism. Neonatal HPT may present in newborns and infants with life-threatening hypercalcemia due to parathyroid hyperplasia. Some of these pediatric patients have multiple endocrine neoplasia syndrome or familial isolated PHPT.

A: Frontal hand radiograph reveals coarsening of the trabecular pattern and signs of bone resorption such as cortical tunneling straight arrows and acroosteolysis curved arrow. The imaging modality of choice for evaluating bone involvement in children with PHPT is radiography. Typical radiographic findings include osteopenia, coarsening of the trabeculae, subperiosteal bone resorption, and cortical tunneling Fig. Subligamentous bone resorption is also common, more typical in the distal femur and proximal tibia. Metastatic soft tissue and visceral calcifications, chondrocalcinosis, slipped capital femoral epiphyses, and bone deformities i.

In cases of confirmed single parathyroid adenoma, exploration and excision of the single affected gland can be performed. Tubular dysfunction causes hypocalcemia because of deficient vitamin D synthesis, whereas impaired glomerular function causes phosphorus retention.

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This results in hypocalcemia that triggers hyperparathyroidism in an effort to restore calcium levels. Secondary hyperparathyroidism is the major metabolic abnormality in ROD. As the disease progresses, rickets and osteomalacia develop.

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Classic radiographic features include subperiosteal, endosteal, and subligamentous resorption as well as osteopenia with coarsening of the trabecular pattern. Subperiosteal resorption is most frequently seen in the middle phalanges of the hand Fig. Endosteal resorption, also known as cortical tunneling, is seen as a lacy pattern of the inner cortex of the bone. Subchondral resorption that is adjacent to the physeal cartilage, the so-called osteitis fibrosa, can also be seen causing lucency at the edge of the physis, simulating rickets and predisposing to epiphyseal slippage.

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Osteosclerosis also is a common manifestation that causes diffuse chalky bone density, which in the spine affects the vertebral end plates, producing the rugger jersey spine. Control of bone and mineral homeostasis is very challenging in children, with most of the affected patients experiencing signs of hyperparathyroidism to different extents. The treatment consists of dietary counseling, ergocalciferol and cholecalciferol supplementation, and the use of calcium-free phosphate binders.

The diagnosis of this entity is mainly based on laboratory findings. Although the characteristic autonomic function of the parathyroid glands in THPT may resemble the metabolic activity of an adenoma, this pathologic entity is in fact rare in THPT. It can be associated with several syndromes, including DiGeorge syndrome, which is characterized by congenital absence of the parathyroid glands. Other causes of HP are autoimmune disorders and postsurgical resection. Radiographic findings include thickening of the cranial vault and facial bones, sutural diastasis in cases with associated increased intracranial pressure, and abnormal tooth development with delayed eruption or supernumerary teeth.

Other manifestations include premature epiphyseal fusion, dense metaphyseal bands, and calcifications in the soft tissue, tendons, and spinal ligaments. A: Frontal hand radiograph shows diffuse osteopenia and signs of bone resorption such as cortical tunneling straight arrows and acrosteolysis with tuft resorption curved arrows. B: Frontal chest radiograph demonstrates resorption of the distal clavicles and scapular tips straight arrows and widening of the costochondral junctions curved arrows.

Note calcified thrombus of the superior vena cava due to previous dialysis catheters arrowhead. C: Chronic kidney disease-mineral bone disorder in an year-old girl with renal failure. A typical radiographic finding in PHP and PPHP is the shortening of one or more of the metacarpals and metatarsals due to the premature fusion of the physis, most commonly affecting the fourth and fifth rays. Other skeletal features of PHP and PPHP include diaphyseal exostoses, coxa valga or vara Schematic I , osseous bowing, cone epiphyses, and accelerated skeletal maturation. Soft tissue calcification similar to HP can also be seen.

For nonresponding cases, PTH replacement therapy can be used; however, the use of recombinant human PTH is contraindicated in children because of the increased risk of osteosarcoma. This eventually leads to the excessive accumulation of inorganic pyrophosphate, resulting in deficient bone mineralization.

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  • The four main types of hypophosphatasia are perinatal, infantile, childhood, and adult types. Similar to rickets, the diagnosis of hypophosphatasia can be made based on radiographs.

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    In hypophosphatasia, there is lack of mineralization of parts of the skeleton which is more severe than the mineralization defect noted in osteogenesis imperfecta. The infantile and childhood forms resemble rickets with less uniform and patchier metaphyseal involvement. The adult form simulates osteomalacia with a coarse trabecular pattern, metatarsal stress fractures, and Looser zones. Lateral radiograph of the thoracolumbar spine shows sclerosis of the vertebral endplates arrowheads with multilevel intervertebral disk calcification foci arrows.

    No effective treatment currently exists for hypophosphatasia. There is only treatment for specific symptoms and supportive measures. Humans are dependent on the exogenous intake of vitamin C that is present in vegetables and fruits. Vitamin C is also present in breast milk. Infantile scurvy usually develops after 6 months of age because of the storage of vitamin C built up in utero and during the first few months in life through breast milk. As the disease progresses, affected pediatric patients develop gum bleeding and bone changes. Skin manifestations and bleeding are more common in older children and adults.

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    Biopsy may occasionally be pursued Fig. Extensive resorption of cortical and cancellous bone predisposes these areas to fracture Fig. The Trummerfeld zone is a disrupted metaphyseal zone of osteoporosis that can result in peripheral metaphyseal excrescences if there is associated healing Fig. The Wimberger ring sign is characteristic of the disease and consists of a radiodense line at the edge of an ossification center that corresponds to the normal mineralized cartilaginous zone of provisional calcification, which contrasts more with the demineralized remainder of the center Fig.

    Alternating dense and lucent metaphyseal lines can be present along with hemarthrosis and subperiosteal hemorrhage, which is seen as periosteal reaction along the long bones. Frontal hand radiograph shows a short fourth metacarpal asterisk. The treatment of scurvy includes vitamin C supplementation, which typically leads to a rapid resolution of the symptoms. B: Scurvy in a 6-year-old boy. Frontal knee radiograph shows a linear metaphyseal lucency with an associated fracture and a focal bony excrescence indicating early healing Trummerfeld zone curved arrow.

    Significant osteopenia, more conspicuous in the epiphysis with a peripheral sclerotic line Wimberger ring sign , is seen asterisks. HM are toxic because of potential cumulative deleterious effects that can cause chronic degenerative changes, especially in the nervous system, liver, kidney, and bones. In children, the mechanism of poisoning is accidental ingestion and most frequently occurs in toddlers. The primary route of lead exposure is through the GI tract, where lead is taken up at calcium absorption sites, which are very active at times of rapid growth.

    Being an inorganic compound, it is poorly absorbed through the GI tract. Chronic exposure to very small amounts of lead is necessary to cause untoward effects. Lead-based paints in households and toys are common causes of lead intoxication in children. Other symptoms of lead toxicity include anemia, anorexia, abdominal pain, vomiting, and growth delay. Pediatric patients in high-risk areas should be screened every 1 to 2 years. The skeletal manifestations of lead poisoning can be evaluated using radiographs. Typical features include dense transverse metaphyseal lines due to the increased deposition of calcium along with lead, most prominently around the knee.

    Dense bands in the femur and tibia can be seen in normal children; thus, the presence of fibular bands supports the diagnosis Fig. Radiodense bands can be seen in other sites of the axial and appendicular skeleton, including the spine. If lead exposure is interrupted, these dense bands slowly migrate to the diaphysis and disappear in a few years.


    Dense metaphyseal bands are not characteristic of lead poisoning because they can be seen with other types of HM intoxication, scurvy, rickets, and treated leukemia. Abnormal tubular bone remodeling, most pronounced in the distal femur with metaphyseal widening as well as sutural widening in the skull due to increased intracranial pressure, can be seen. Frontal tibia and fibula radiograph shows a typical dense metaphyseal band lead band arrows around the knee and ankle.

    The proximal fibular involvement is more supportive of the diagnosis of lead poisoning. All symptomatic and asymptomatic pediatric patients with markedly elevated blood levels of lead should be handled as emergencies and treated immediately with chelating agents.

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    Chronic manifestations are mainly related to osteonecrosis and secondary bone collapse and deformity. Vasoocclusive crisis affects virtually all pediatric patients with SCD, often beginning in late infancy and recurring throughout their lives. The pathogenesis of vasoocclusive crisis relates to microvascular occlusion due to the abnormally deformed red cells that may be seen in any organ but occur more frequently in the bone marrow.

    These microvascular occlusions result in bone marrow infarction, typically in the medullary cavity or epiphyses. The vertebral column shows large areas of medullary pallor infarcts as well as extensive vertebral body collapse. Clinically, affected pediatric patients complain of localized or multifocal intense pain, edema and erythema with or without fever and leukocytosis. Affected children present with a swollen and painful digit. Radiographs are of little value, as the acute changes are usually not seen. Bone scintigraphy or MRI is the imaging modalities of choice during an acute pain crisis.

    Bone scintigraphy is helpful in assessing multifocal involvement showing increased uptake in areas of acute bone marrow infarction. In the acute setting, bone infarcts are hyperintense on fluid-sensitive sequences and hypointense on T1-weighted MR images. On T1-weighted MR images with fat saturation before contrast, acute lesions tend to be hyperintense and display no enhancement after contrast. When epiphyseal in location, infarcts can be associated with joint effusion. Adjacent soft tissue edema and stranding are findings that support a more acute process.

    The more chronic infarcts tend to be predominantly hypointense with a peripheral bright halo on fluid-sensitive sequences and hypointense with fatty elements on T1-weighted MR images Fig. SCD-related bone marrow infarcts tend to be larger than those from other etiologies, reflecting a more systemic disease. More chronic infarcts can be seen on radiographs as serpiginous lytic lesions with a peripheral sclerotic line Schematic C. On radiographs, diffuse or patchy osteosclerosis is also present Fig.


    In the epiphyses, a subchondral crescentic lucency is fairly characteristic on radiographs Schematic F. The oblique radiograph of foot shows marked periosteal reaction arrows and adjacent soft tissue swelling involving the first and second metatarsals. The treatment of a vasoocclusive crisis and infarcts from SCD requires the use of analgesics, vasodilators, and aggressive intravenous hydration.

    If a coexisting infection is suspected, antibiotics are also given. A: Coronal T1-weighted MR image of the pelvis shows bilateral proximal femoral serpiginous lesions with fatty elements and a sclerotic low-signal rim indicative of old bone infarcts asterisks. There is a decreased T1 signal in both proximal femoral diaphyses indicative of bone marrow reconversion arrows.

    B: Frontal radiograph of the pelvis demonstrates patchy increased sclerosis asterisks in both iliac wings and femoral heads indicating multiple previous bone infarcts. The femoral heads are symmetric in size and shape with preserved sphericity. Storage Diseases Storage diseases are progressive multisystemic metabolic conditions that affect the musculoskeletal system and viscera, such as the lungs, heart, liver, spleen, and in some cases the brain as well. They can be mainly divided into lysosomal and nonlysosomal diseases, with the metabolic substrate determining the involvement of the cell type or organ in the individual storage disease.

    Most LSDs are inherited as an autosomal recessive pattern. Typically, LSDs are classified based on the accumulating substrate: mucopolysaccharidoses MPS , mucolipidoses, sphingolipidoses, glycoprotein storage diseases, and glycogenosis. LSDs are differentiated by clinical features, the age of presentation, and enzyme deficiency. All LSDs affect the skeletal system with overlapping phenotypes; however, some have unique skeletal involvement. In the MPS, the accumulation of glycosaminoglycans GAGs occurs because of a deficiency in different GAG-degrading enzymes, thus causing progressive damage of affected tissues.

    Seven distinct clinical types of MPS have been identified, caused by 11 different enzymatic deficiencies Table Weisman, M. Handbooks in Health Care Co. An explosion of new information about the pathophysiologic processes involved in diseases of the respiratory system of the newborn has led to improved treatments, such as surfactant replacement, that have markedly reduced morbidity and mortality in this vulnerable population.

    The third edition of this book reviews the epidemiology, pathophysiology, presentation, management, and outcomes for respiratory disorders affecting the newborn, and has updated reviews of pulmonary physiology, ventilatory and non-ventilatory management of respiratory distress. Supportive care that is necessary to optimize outcome is emphasized through sections on nutritional and gastrointestinal support, communications with parents, ethical consideration, and discharge planning. An unparalleled documentation of the illnesses and abnormalities that can afflict the newborn compiled over the year career of pediatrician and neonatologist, Dr.

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    Atlas of the Newborn Arnold J. Rudolph, M. Volume 1. Neonatal and perinatal medicine Volume 2.